General Dermatology Flashcards

Question

25- What is the most common area affected in multifocal fixed drug eruption? A. Oral or genital mucosa B. Central face C. Upper arms D. Thighs E. Lower abdomen

Answer 1

Correct choice: A. Oral or genital mucosa Explanation: Over half of FDE lesions occur in the oral or on the genital mucosa.

Answer 2

Correct choice: A. Gold Explanation: Gold is known to cause a delayed patch test reading, with some recommending a final patch test reading at 3 weeks to avoid missing any positive reactions. Patients allergic to gold often also react to nickel and cobalt. Bacitracin is a frequent contact allergen that often coexists with an allergy to neomycin. Fragrance mix contains cinnamic alcohol and aldehyde, hydroxycitronellel, isoeugenol, eugenol, oak moss absolute, alpha-amyl cinnamic aldehyde, geraniol. Rosin (colophony) is found in adhesive tape, cosmetics, glossy papers, violin bows, and chewing gum.

Answer 3

Correct choice: A. Start Differin 0.1 gel nightly to every other night after wash Explanation: The patient has comedonal acne. She should wash her face 2 times a day with a gentle cleanser. She should start using Differin 0.1 gel nightly to her face after washing. If too drying, the patient can reduce usgae of Differin to every other night. A topical retinoid treats comedones and assists with the proper keratinization of the hair follicle. Aggressive exfoliation can damage the skin and make acne worse. Doxycycline is a systemic treatment option in cases of inflammatory acne that has not responded to combined topical treatment with clindamycin/benzoyl peroxide in the AM and a topical retinoid in the PM. Monotherapy with clindamycin is ill advised in cases of inflammatory acne as bacterial resistance becomes a problem; the patient should simultaneously used a benzoyl peroxide product. Isotretinoin is the not the first treatment in cases of comedonal acne.

Answer 4

Correct choice: A. Sneddon Wilkinson disease Explanation: Sneddon Wilkinson is characterized by annular or polycyclic lesions, usually commencing in the flexures. Very superficial (subcorneal) sterile pustules are the hallmark of Sneddon–Wilkinson disease. There may be a gravity-induced demarcation in some vesiculopustules, with clear fluid superiorly and pus inferiorly. This disease has a cyclic course, i.e. as the pustules resolve they are replaced by superficial scaling and then new pustules form again. Some patients with Sneddon–Wilkinson disease have an associated IgA paraproteinemia. Its response to dapsone, combined with subcorneal pustules (in the absence of spongiform pustules), provide support for this condition being a disease entity distinct from pustular psoriasis. Pustular psoriasis: In generalized pustular psoriasis, infiltration of neutrophils dominates the histologic picture and the clinical picture consists of sterile pustules on a background of bright erythema. Acute generalized exanthematous pustulosis: This condition would present with mild spongiform pustulation, eosinophils and often a drug history with either a new medication or a change in drug history. Superficial folliculitis: The clinical presentation and distribution would differ as superficial folliculitis is clinically characterized with appearance of pustules at follicular orifices and in infundibulum. Benign familial pemphigus is an uncommon autosomal dominant disorder clinically characterized by flaccid blisters and erosions seen in intertriginous areas, especially the axillae and groin; moist, malodorous vegetations and fissures can develop.

Answer 5

Correct choice: c. Smoking is significantly related to these findings and cessation may be helpful Explanation: Palmoplantar pustulosis, seen and descibed here, had been linked to smoking and smoking cessation can lead to improvement in some patients. The other options are not true regarding palmoplantar pustulosis.

Answer 6

Correct choice: D. Trichorhinophalangeal syndrome Explanation: Trichorhinopharyngeal syndrome is the only condition from the mentioned conditions that is not associated with saddle nose deformity.It is associated with sparse hair, a bulbous (pear- shaped) nose and cone-shaped epiphyses. The other listed conditions can result in saddle nose deformity.

Answer 7

Correct choice: D. Discuss medication usage patterns Explanation: Considering high rates of medication nonadherence in acne, it is most appropriate to review a patient’s usage patterns and address factors limiting use before altering therapy. Using a common clinical scenario, this question emphasizes to the examinee the need to assess medication adherence prior to changing therapy. A wide variety of patient-, therapy-, and healthcare system-associated factors influence treatment adherence. Therefore, it is most appropriate to first discuss with the patient how (or if) they are using their medication (choice 4) in order to discover any barriers, real or perceived, to optimal usage. Commonly, irritancy limits use of topical acne products, so it would be inappropriate to increase retinoid strength (choice 1) without first reviewing actual use. A short course of oral antibiotics (choice 2) or addition of a topical antibiotic (choice 5) may be appropriate measures if the patient is first determined to be using the prescribed topical product as intended. Isotretinoin (choice 3) is indicated for severe, recalcitrant nodulocystic acne and thus would not be appropriate to initiate prior to understanding how the patient is using their topical product and considering the full range of available treatment options.

Answer 8

Correct choice: D. Prednisone Explanation: The clinical presentation most likely represents Sweet syndrome (acute febrile neutrophilic dermatosis). Classical Sweet syndrome is frequently associated with recent infections, IBD and pregnancy. Diagnosis is made with 2 major and 2 minor criteria. Major criteria: 1. Abrupt onset of painful erythematous plaques 2. Histopathological evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis Minor criteria: 1. Fever 2. Association with malignancy, inflammatory disease, pregnancy or recent URI, GI infection, vaccination 3. Excellent response to systemic steroids 4. Abnormal lab values (ESR, CRP, leukocytosis, >70% neutrophils)

Answer 9

Correct choice: A. Koebnerization Explanation: Koebnerization is seen in several skin conditions including lichen nitidus, seen here. It is also referred to as an isomorphic response. The skin condition can grow within areas of trauma, as seen by the linearity and nearby scratches in this photo. The other options are not seen with lichen nitidus.

Answer 10

Correct choice: A. Observation Explanation: Lichen nitidus, seen here, is a benign often self-resolving dermatosis. Treatment is not needed, particularly if asymptomatic, and observation is appropriate. Topical steroids and calcineurin inhibitors can be used if lichen nitidus is pruritic, otherwise no treatment is needed. Oral acitretin and topical vitamin A medications are unlikely to be helpful.

Answer 11

Correct choice: B. Methylchloroisothiazinolone Explanation: Methylchloroisothiazinolone, also known as Kathon CG, is the only non- formaldehyde releasing preservative. It is found in cosmetics, skin/hair products, "acid" permanent waves, soaps, latex emulsions, and biocides. All the others are formaldehyde-releasing preservatives.

Answer 12

Correct choice: B. Neutrophilia Explanation:Acute generalized exanthematous pustulosis is often seen with fever and neutrophilia and can also have facial edema. Renal, bone marrow, neurologic and pulmonary involvement are rare.

Answer 13

Correct choice: C. Reassurance Explanation: The image depicts piezogenic papules, a benign entity associated with fatty connective tissue protrusion (most commonly) of the heel. Plain film of the foot will not reveal anything. Magnetic resonance imaging of the foot may provide additional detail particularly if the foot were under pressure - revealing areas of slight protrusion of fatty connective tissue - but imaging is not necessary to make this diagnosis. Punch biopsy and tissue culture are unnecessary and will do more harm than good.

Answer 14

Correct choice: D. Löfgren syndrome Explanation: Löfgren syndrome is associated with a favorable prognosis in sarcoidosis. This question tests the examinee’s knowledge of the types of cutaneous sarcoidosis and their associated prognoses. Of the answer choices, Löfgren syndrome (choice 4), which includes erythema nodosum, hilar adenopathy, arthritis, and fever, tends to run the most limited course, resolving spontaneously within 1-2 years. In contrast, lupus pernio (choice 1) is associated with more severe and longstanding systemic sarcoidosis requiring intensive treatment. Blau syndrome (familial juvenile systemic granulomatosis, choice 2) is a dominantly inherited disease that is phenotypically similar to sarcoidosis, manifesting in childhood with granulomatous inflammation of the skin, eyes, and joints. Heerfordt syndrome (uveoparotid fever, choice 3) comprises uveitis, parotitis, fever, and cranial nerve palsy, while Mikulicz syndrome (choice 5) describes involvement of lacrimal, salivary, and parotid glands; neither is as favorable as Löfgren syndrome.

Answer 15

Correct choice: B. 5-15% Explanation: 5-15% of patients with discoid lupus will develop systemic lupus erythematosus.

Answer 16

Correct choice: C. Gastrointestinal involvement portends a poor prognosis Explanation: In Dego's Disease (aka Malignant Atrophic Papulosis), GI involvement portends a poor prognosis as death from this condition is usually due to fulminant peritonitis caused by multiple perforations of the intestines. Dego's Disease (aka Malignant Atrophic Papulosis): It most frequently affects men and is a potentially fatal obliterative arteritis. After undergoing multiple stages, the patient is left with varicelliform scars. Later, anemic infarcts involve the intestines to produce acute abdominal symptoms of epigastric pain, fever, and hematemesis. Lab results indicate a high plasma fibrinogen level and increased platelet aggregation. Administration of corticosteroids has not been beneficial.

Answer 17

Correct choice: C. Medication history Explanation: For a new-onset lichenoid eruption, it is crucial to review the medication history and consider the possibility of lichenoid drug eruption, which can be clinically and histopathologically identical to idiopathic lichen planus (LP). This question tests the examinee’s knowledge of pertinent history to obtain for a new-onset lichenoid eruption. In this scenario, it is essential to review both prescription and nonprescription medication use (choice 3) and consider the possibility of lichenoid drug eruption, which can be clinically and histologically indistinguishable from idiopathic LP. Rather than hepatitis B (choice 1), hepatitis C status is important to determine, more so in specific populations and with oral LP. LP is not related to sexual activity (choice 2). Familial LP (choice 4) is relatively uncommon and less useful to review than medications. As LP is not a paraneoplastic phenomenon, malignancy screening (choice 5) would not be relevant in this situation.

Answer 18

Correct choice: B. Calciphylaxis Explanation: Calciphylaxis begins as fixed livedo reticularis (livedo racemosa), which is frequently firm or hard to the touch. Areas within the livedo become increasingly violaceous and eventually purpuric, bullous, and necrotic. Affected tissue has reduced oxygenation. Lesions affect the legs below the knees in 90% of patients. More proximal lesions and those of the fatty areas of the thighs, buttocks, and abdomen occur in about two-thirds. Severe pain is a cardinal feature of calciphylaxis, often requiring narcotic analgesia for control. 1 - Livedo racemosa refers to a form of LR that has a larger, branching, and more irregular pattern and is often more widespread, affecting both the extremities and the trunk 3 - Cryoglobulins are cold-precipitable immunoglobulins that can be divided into three subtypes, all of which have cutaneous manifestations including palpable purpura, myalgias and arthralgia, peripheral neuropathy and glomerulonephritis. 4 - Disseminated intravascular coagulation is a condition in which small blood clots develop throughout the bloodstream, blocking small blood vessels. The increased clotting depletes the platelets and clotting factors needed to control bleeding, causing excessive bleeding. 5 - Hematoma is a solid swelling of clotted blood in the tissues

Answer 19

Correct choice: C . Associated with premature labor Explanation: The figure illustrates polymorphic eruption of pregnancy (PUPPP) with erythematous papules within striae. There is no associated maternal or fetal risk. All of the remaining choices are true.

Answer 20

Correct choice: B. RPR Explanation: Patient has moth-eaten alopecia of secondary syphilis. The correct answer is RPR to screen for syphilis. Copper and zinc levels are not necessary. The hair loss is not consistent with androgenetic alopecia, but even if it were you do not need free testosterone and total testosterone levels to make that diagnosis. ESR and CRP are non-specific tests to evaluate for inflammation in the body and are not useful in this setting.

Answer 21

Correct choice: E. Doxycycline Explanation: This is peri-oral dermatitis, which is best treated with doxycycline of these choices. Several therapies, including oral antibiotics (e.g., tetracyclines), topical antibiotics (e.g., erythromycin, clindamycin), topical metronidazole, topical azelaic acid, topical sulfacetamide- sulfur, and topical calcineurin inhibitors (e.g., tacrolimus, pimecrolimus), have been used with some success to treat peri-oral dermatitis. Brimonidine gel and oxymetazoline cream are used to treat the persistent erythema of rosacea. While many believe that peri-oral dermatitis may represent a form of rosacea, brimonidine gel and oxymetazoline cream will not treat the papules and pustules seen in the image here. Hydrocortisone ointment (and other topical steroids) typically worsen peri-oral dermatitis and thus should be avoided. Ketoconazole cream is a topical anti-fungal that does not treat peri-oral dermatitis.

Answer 22

Correct choice: D. Diabetes Explanation: Granuloma annulare is a benign, non-infectious, granulomatous condition of the skin that is usually asymptomatic and self-limited. It typically presents with annular erythematous plaques that are most commonly found on the dorsal surfaces of the hands and feet. Generalized and perforating variants are most closely associated with diabetes.

Answer 23

Correct choice: B. Scrubbing with an alcohol swab Explanation: This is an example of terra firma-forme or Duncan's dirty dermatosis. It presents as dirt-like plaques and is both diagnosed and treated with isopropol alcohol. The other options have not been shown to help treat terra firma-forme.

Answer 24

Correct choice: D. Start mometasone 0.1 ointment BID to affected areas on body, advise face to feet moisturizer at least BID Explanation: The correct answer is to begin topical steroid two times a day to the rash and use emollient at least two times a day to the body in patient with 8% BSA psoriasis who has not tried any prescription treatments. While the other options are treatments that could be used in for psoriasis in a patient with HIV and psoriasis, it is most appropriate to start with topical steroid and emollient for treatment. Cyclosporine is not a chronic medication for psoriasis and is not a medication to use for 8% BSA psoriasis.

Answer 25

Correct choice: B. Chigger bites Explanation: The patient has encountered chiggers, mite larvae who feed on human keratinocytes. Their feeding process induces the development of the characteristic chigger bite, which is very pruritic. Topical steroids can reduce the itch sensation. Dermatitis herpetiformis commonly presents on extensor surfaces as pruritc papules and vesicles with excoriation. The onset would not be as sudden and limited to the feet. The lesions are not excoriations (a secondary skin lesion) and thus neurotic excoriations is incorrect. Allergic contact dermatitis would present as ill-defined eczematous pink patches with scale, not as discrete papules. Porphyria cutanea tarda would show evidence of vesicles, scars, and milia - none of which are present in this photograph. In addition, the condition is chronic.

Answer 26

Correct choice: A. Actinic keratosis Explanation: Actinic keratosis can develop into squamous cell skin cancer, thus it is a pre-cancerous lesion. Seborrheic keratosis is a benign thickening of the skin not associated with a pre-malignant states. Onychomycosis is a fungal infection of the nail not associated with a pre-malignant state. Psoriasis is an inflammatory skin condition not associated with a pre-malignant state. Impetigo is a superficial infection of the skin by bacteria and is not associated with a pre-malignant state.

Answer 27

Correct choice: B. Lichen sclerosus Explanation: Autoantibodies to extracellular matrix protein-1 (ECM-1) are found in lichen sclerosus (LS). This question tests the examinee’s knowledge of the pathogenesis of LS. In LS (choice 2), up to 80% of patients have circulating autoantibodies to ECM-1, a glycoprotein that contributes to integrity of the basement membrane zone and assembly of collagen fibrils. An important distinction needs to be made between LS and lipoid proteinosis (choice 1), in which there is an ECM1 gene mutation. The remaining answer choices are caused by distinct gene mutations: PORCN in focal dermal hypoplasia (Goltz syndrome, choice 3); IKBKG/NEMO in incontinentia pigmenti (choice 4); and fibrillin 1 in Marfan syndrome (choice 5).

Answer 28

Correct choice: A. Chronic lymphocytic leukemia Explanation: Patients with chronic lymphocytic leukemia may develop exaggerated reactions to insect bites, including bullous reactions. Patients with the other listed conditions to do exhibit exaggerated reactions to insect bites.

Answer 29

Correct choice: B. Gold sodium thiosulfate Explanation: Several studies have found gold to be the most common relevant culprit in eyelid allergic contact dermatitis (ACD). This question assesses the examinee’s knowledge of ACD involving the eyelids. Common eyelid contact allergens include metals (most prominently gold [choice 2], nickel, and cobalt), fragrances, preservatives, topical antibiotics, and surfactants. None of the other options are among the most common causes of eyelid ACD: budesonide (choice 1) is the screening agent for group 1 topical corticosteroids; thimerosal (choice 3) is a preservative that uncommonly causes ACD (although it is found in some ophthalmic drops); epoxy resin (choice 4) is found in adhesives; and paraben mix (choice 5) screens for parabens, common preservatives in cosmetics and personal care products that rarely cause ACD.

Answer 30

Correct choice: B. Segmental vitiligo is significantly increased in adults compared to children Explanation: Segmental vitiligo is significantly increased in children compared to adults. Although vitiligo vulgaris is the most common clinical type observed in children, the frequency of segmental vitiligo (~15–30%) is significantly increased compared to that in adults (<5–10%). The incidence of associated endocrinopathies is less than in the adult vitiligo population. A family history of vitiligo is associated with an earlier age of onset. - Vitiligo vulgaris is the most common clinical type of vitiligo observed in children - In lesions of vitiligo, melanocytes are typically absent or present in small numbers. The epidermal melanocyte density can be assessed with melanocyte-specific immunohistochemical stains, such as Melan-A (MART-1), MITF, and HMB45, or via incubation of biopsy specimens with dihydroxyphenylalanine (DOPA; detects tyrosinase activity). Although ultrastructural studies may be performed for research purposes, very few hypomelanotic disorders have specific ultrastructural findings.

Answer 31

Correct choice: E. Chronic irritation from biting Explanation: Morsicatio buccarum is the term describing a shaggy white plaque on the buccal mucosa occurring secondary to chronic irritation from biting.

Answer 32

Correct choice: C. Oral doxycycline Explanation: The patient has rosacea, a chronic inflammatory rash classically of the mid face. Rosacea does not spare the nasolabial folds, while cutaneous lupus does. Lupus spares the nasolabial fold. In the case of lupus, you would consider ordering ANA, C3, and C4 and you may start prednisone if the patient is having disease severe enough to warrant treatment with systemic steroid. Ordering rheumatoid factor, ESR, and CRP will not help the patient and oral acyclovir is not indicated as the patient does not have a viral disease such as herpes simplex or herpes zoster.

Answer 33

Correct choice: C. Pityriasis rubra pilaris Explanation: Pityriasis rubra pilaris characteristically presents with follicular papules with an erythematous base, including on the proximal dorsal fingers. There is a coalescence of orange–red plaques, but with obvious islands of sparing. An orange–red waxy keratoderma of the palms and soles is often seen. 1 - Seborrheic dermatitis: Early PRP of the scalp can mimic seborrheic dermatitis. As additional clinical features of PRP develop, these two entities can be distinguished. PRP has additional distinguishing features such as follicular papues, orange-red plaques, islands of sparing, and waxy keratoderma. Scalp dermatitis is much more responsive to conventional therapy. The relative recalcitrant nature of scalp PRP may serve as a clue to the diagnosis. 2 - Cutaneous T-cell lymphoma: A PRP-like eruption can be seen in patients with dermatomyositis (Wong type) and cutaneous T-cell lymphoma, as can erythema and scaling of the scalp however the other clinical findings are characteristic of pityriasis rubra pilaris. 4 - Kawasaki disease: Children with acute-onset PRP may be misdiagnosed as having Kawasaki disease, however the older age of this patient and the other associated clinical features present do not support this diagnosis. 5 - Psoriasis: The major entity in the differential diagnosis of pityriasis ruby pillars is psoriasis. The distinctive orange–red palmoplantar keratoderma plus the keratotic follicular papules with a nutmeg grater appearance, the classic islands of sparing of the trunk, a finer scale, and no family history of psoriasis help differentiate PRP from psoriasis. The presence of oil-drop changes, small pits, and marginal onycholysis of the nails favor psoriasis.

Answer 34

Correct choice: B. White dermatographism Explanation: The photo is of Dennie-Morgan lines in a chronic atopic dermatitis patient. Regular nail pitting is seen in psoriasis; atopic dermatitis has increased cutaneous infections; scarring hair loss and inflammatory arthritis are not associated with atopic dermatitis.

Answer 35

Correct choice: D. Squamous cell carcinoma is a potential sequela Explanation: This is an image of discoid lupus erythematosus (DLE). Squamous cell carcinoma may develop from lesions of DLE. SCCs arising from lesions of DLE are rare and aggressive tumors with greater likelihood of metastases. Cases have been reported among patients with different clinical characteristics. Classically, 5-15% of patients with DLE will eventually develop SLE. C2 (not C3) deficiency increases susceptibility to autoimmune conditions, particularly lupus (DLE, SCLE, SLE). Biopsy characteristically shows a thinned epidermis with plugged follicles, vacuolar degeneration of the basal layer, a thickened basement membrane, melanin incontinence, perivascular/periadnexal lymphocytes, and increased mucin deposition between collagen bundles. UV exposure exacerbates DLE, so UV protection is a critical aspect of treatment.

Answer 36

Correct choice: D. Pyogenic granuloma Explanation: A pyogenic granuloma is one of several vascular lesions that can present on the skin during pregnancy, and they often develop rapidly in a peri-ungual location. At present, the pathogenesis of pyogenic granuloma is unknown. In addition to pregnancy, reported triggers include antecedent trauma and certain drugs, including acitretin, HIV protease inhibitors, EGFR inhibitors, docetaxel, capecitabine and rituximab. In the case of pyogenic granulomas arising during pregnancy, they often regress after delivery and treatment is not usually required unless they cause excessive bleeding. Other vascular lesions that can develop during pregnancy include palmar erythema, spider angiomas, varicose veins, cavernous hemangiomas, glomus tumors, and hemorrhoids. The development of vascular lesions during pregnancy is thought to be due to a combination of increased hormones and increased intravascular pressure. Of the answer choices listed, a pyogenic granuloma is the most likely diagnosis based on the clinical description.

Answer 37

Correct choice: A. Propylthiouracil Explanation: PTU is well-reported to cause p-ANCA positive vasculitis.

Answer 38

Correct choice: D. Halobetasol 0.05% ointment 2 x a day to areas with rash Explanation: The patient has psoriasis with BSA 8% with no treatment trial previously. The most reasonable next step is to begin a potent topical steroid on the areas with psoriasis on the legs 2 x a day. You can consider occlusion of the steroid at night if patient not responsive to topical steroid or if the steroid tends to rub off at night onto the bed. The patient does not have a BSA% greater than 10 and thus choices such as Tremfya and Remicade are an over-treatment in this patient who has not trialed any conservative measures yet. If patient had psoriatic arthritis, you should then entertain biologic treatment but not for psoriasis vulgaris with BSA 8% on the legs. nbUVB to the whole body 3 x a week is over-treatment for a patient with such limited skin disease of 8% on the legs. You could consider Excimer treatment (handheld nbUVB targeted treatment for smaller disease areas on the skin) if you had the device in your office but that was not one of the choices. Much like Tremfya and Remicade, Otezla would represent an over-treatment for the patient in the vignette but you could consider using the medication if the patient also had psoriatic arthritis.

Answer 39

Correct choice: D. Ustekinumab Explanation: This patient has psoriasis, psoriatic arthritis, and Crohn’s disease. Ustekinumab binds the p40 subunit to inhibit IL-12 and IL-23. Ustekinumab is approved for the treatment of plaque psoriasis, psoriatic arthritis, and Crohn’s disease. It is the best treatment choice. A higher dose of ustekinumab is needed to treat Crohn’s disease than to treat psoriasis. Secukinumab is a monoclonal antibody that inhibits IL-17A. It is FDA-approved to treat psoriasis and psoriatic arthritis, but like other IL-17 inhibitors (e.g. Ixekizumab) should not be used in Crohn’s disease. Guselkumab inhibits IL-23 by selectively binding the p19 subunit of IL-23. It was FDA-approved in July 2017 to treat plaque psoriasis only. Apremilast is approved for the treatment of psoriasis and psoriatic arthritis, but not Crohn's disease (thereby making Ustekinumab the better choice).

Answer 40

Correct choice: D. Contact dermatitis Explanation: This periumbilical eczematous eruption is classic for allergic contact dermatitis secondary to nickel exposure. The metal snaps on the pants are the cause of the nickel exposure in this case. The remaining answer choices are less likely, especially given that the image shows a well-demarcated eczematous periumbilical eruption and metal snaps suggesting a contact nickel allergy.

Answer 41

Correct choice: B. White gold Explanation: The case presented is consistent with a contact dermatitis, likely allergic contact dermatitis to a metal. The most common allergen is nickel. White gold may contain nickel, therefore this type of metal should be avoided. Patient's allergic to nickel, typically can tolerate gold 18 ct or higher, stainless steel, sterling silver, and pure platinum.

Answer 42

Correct choice: C. Granuloma annulare Explanation: The dermatosis seen here is annular, with erythematous and raised borders and is slightly pruritic. Of the choices, granuloma annulare is the most likely diagnosis. The alternative options do not represent the clinical granulomatous disease of granuloma annulare.

Answer 43

Correct choice: A. Prednisone Explanation:Prednisone is the most appropriate initial treatment for neutrophilic dermatosis of the dorsal hands. The examinee is presented with the image of an eroded violaceous plaque on the dorsal hand that is highly consistent with neutrophilic dermatosis of the dorsal hands, an entity combining clinical and pathologic features of Sweet’s syndrome and pyoderma gangrenosum. Prednisone (choice 1) is the most appropriate first-line treatment. While the remaining options (choices 2-5) have been used as second-line and beyond treatment of Sweet’s syndrome and/or pyoderma gangrenosum, none of them represents the most appropriate first-line therapy.

Answer 44

Correct choice: C. Post-operative follow-up of a full thickness skin graft with a bolster Explanation: Hydrogen peroxide is cytotoxic and therefore not a first choice treatment in cleaning chronic wounds and ulcers. Hydrogen peroxide is use to remove crusts on healing wounds such as bolster on a skin graft.

Answer 45

Correct choice: E. Serum protein level Explanation: Mortality from Stevens-Johnson Syndrome/toxic epidermal necrolysis is predicted by the SCORTEN scoring system. Prognostic factors include age, heart rate, presence of cancer/ malignancy, BSA involved > 10% on day 1, serum urea level, serum glucose level, and serum bicarbonate level.

Answer 46

Correct choice: D. Nasopharyngeal carcinoma Explanation: Muscle weakness coupled with erythematous papules overlying the dorsal hand joints (Gottron's papules) is highly suggestive of dermatomyositis, which can be a paraneoplastic phenomenon. Nasopharyngeal carcinoma, one of the most common cancers in southern China and Southeast Asia, was the most common associated cancer in Asian studies of dermatomyositis. The remaining malignancies may produce dermatomyositis as a paraneoplastic phenomenon, but they are less likely in a man originally from southern China.

Answer 47

Correct choice: E . Male gender Explanation: Known risk factors for calciphylaxis include end-stage renal disease, hemodialysis, obesity, diabetes mellitus, and female gender as well as warfarin and liver disease. Hyperparathyroidism, calcium supplementation, and vitamin D intake are additional risk factors, with the latter possibly related to the differentiation of vascular smooth muscle cells into osteoblast- like cells. Known risk factors for calciphylaxis include end-stage renal disease, hemodialysis, obesity, diabetes mellitus, and female gender as well as warfarin and liver disease.

Answer 48

Correct choice: D. Idiopathic Explanation: Scrotal calcinosis, or “idiopathic calcified nodules of the scrotum,” cannot be attributed to any identifiable local or systemic causes. This question assesses the examinee’s ability to recognize scrotal calcinosis and discriminate between the five main types of cutaneous calcification (calcinosis cutis). Scrotal calcinosis is a common form of idiopathic calcification (choice 4), meaning that it is not related to any recognizable local or systemic etiologies. In contrast, metastatic calcification (choice 1) affects normal tissue during states of calcium/phosphate dysregulation, most commonly renal disease. Dystrophic calcification (choice 2) occurs when there is local tissue damage in the setting of normal calcium metabolism, taking place in a broad range of conditions including autoimmune connective tissue diseases (CREST syndrome, childhood dermatomyositis), lobular panniculitides (especially pancreatic), traumas (neonatal heel sticks), genodermatoses (pseudoxanthoma elasticum), infections (parasitic primarily), and neoplasms (pilomatricomas). Iatrogenic calcification (choice 3) is induced by diagnostic or therapeutic interventions, most often by extravasation of calcium-containing intravenous solutions. Mixed calcification (choice 5) combines the features of metastatic and dystrophic calcification; calciphylaxis is a prominent example.

Answer 49

Correct choice: B. Herpes gestationis Explanation: Herpes gestationis is also known as gestational pemphigoid or pemphigoid gestationis. It is a rare, pruritic vesiculobullous eruption that develops during late pregnancy or the immediate postpartum period. On direct immunofluorescence, there is linear C3 deposition along the basement membrane zone in 100% of patients (30% of patients also have IgG deposition). IgG1 autoantibodies are directed against a transmembrane hemidesmosomal protein (BP180, BPAG2, collagen XVII). There is an increased risk of prematurity and small-for-gestational age neonates – the risk correlates with the severity of disease. It commonly recurs in subsequent pregnancies. 1 – Prurigo of pregnancy is a benign nonspecific pruritic papular rash that arises during pregnancy. It has also been described with other names such as prurigo gestationis, early onset prurigo of pregnancy, and pruritic folliculitis of pregnancy. It typically manifests as a papular dermatitis (prurigo) and can resemble nodular prurigo. Direct immunofluorescence is negative and histology is nonspecific. It is not associated with any maternal or fetal risks, and typically resolves after delivery of the baby. 3 – Polymorphic eruption of pregnancy manifests as urticarial papules and plaques that usually appear within striae distensae during the latter portion of the third trimester or immediately postpartum. There is development of polymorphous features (vesicles, erythema, target, and eczematous lesions) with disease progression. It is most frequent in primiparous women. Direct immunofluorescence is negative, routine labs are normal, and histologic features are nonspecific. There are no maternal or fetal risks. 4 – Atopic eruption of pregnancy is characterized by eczematous or papular skin lesions in a patient with atopic diathesis in whom other specific dermatoses have been excluded. It generally appears earlier than other pregnancy-related dermatoses (75% before the third trimester). Histology is nonspecific, direct immunofluorescence is negative. Up to 70% of patients have elevated serum IgE levels. There are no maternal or fetal risks. 5 – Intrahepatic cholestasis of pregnancy is characterized by pruritus without primary skin lesions with an onset during the third trimester. Secondary changes correlate with disease duration and vary from subtle excoriations to severe prurigo nodularis. Histology is nonspecific and direct immunofluorescence is negative. Elevated total serum bile acid levels are diagnostic. There is an increased risk of prematurity, intrapartum fetal distress and stillbirths.

Answer 50

Correct choice: B. Thimerosol Explanation: Thimerosol is a preservatives in vaccines such as the influenza, tetanus, and diphtheria vaccines. It is also found in antitoxins and immunoglobulins. Thimerosol is a mercury-containing organic compound. Lanolin is from the sebum of sheep. Ethylenediamine dichloride is a stabilizer in topical creams, medicines, dyes, insecticides, and fungicides and was previously found in nystatin cream. Triclosan is an antibacterial agent found in soap, shampoo and mouthwash. Gluteraldehyde is a cold sterilizing solution used for medical and dental equipment.

Answer 51

Correct choice: D. Septic osteomyelitis Explanation: SAPHO syndrome consists of a spectrum of aseptic neutrophilic dermatoses in association with aseptic osteoarticular lesions that have distinctive histologic and radiographic features. Osteoarticular manifestations include: inflammatory synovitis; arthrosteitis (inflammation of the osseous structures of a joint); hyperostosis or osteitis, most often anterior chest wall (sternum, clavicles, ribs) and axial skeleton (spine, pelvis). A subtype of chronic recurrent multifocal osteomyelitis (CRMO) manifests as frequent involvement of metaphyses of tubular bones and is seen primarily in children.

Answer 52

Correct choice: A. Schamberg disease Explanation: Schamberg disease is the most common form of pigmented purpura. It can occur in children and has a peak frequency in middle-aged to older men. It is characterized by yellow-brown patches with an oval to irregular outline. There are pinpoint peteachiae within patches - likened to cayenne pepper. It occurs in successive crops and favors the lower legs. 2 - Lichen aureus is a rare variant of pigmented purpura which is characterized by a solitary patch or purpuric macules, papules and patches primarily on the lower extremities. 3 - Purpura annularis telangiectoides of Majocchi is uncommon and occurs in adolescents and young adults, especially women. It is characterized by 1-3 cm annular plaques that may slowly expand. There are punctate telangiectasias and petechiae within the border. The location is the trunk and proximal lower extremities. 4 - Pigmented purpuric lichenoid dermatitis of Gougerot and Blum is a rare variant of pigmented purpura that occurs in middle-aged to older men. It is an admixture of two ypes of lesions: Schamberg-like, purpuric red-brown lichenoid papules. It is chronic and occasionally pruritic. 5 - Eczematid-like purpura of Doucas and Kapetanakis is a rare variant of pigmented purpura characterized by scaly petechial or purpuric macules, papules and patches. It is pruritic and mostly on the lower extremities.

Answer 53

Correct choice: D. Presents with a reticulate pattern on the dorsal hands and feet Explanation: Nekam's Disease (keratosis lichenoides chronica) presents with violaceous papules and nodules, hyperpigmented and hyperkeratotic, covered with gray scales. There is often a linear and reticulate pattern on the dorsal hands and feet, extremities and buttocks. This condition is generally very refractory to treatment. The other answer choices listed are incorrect.

Answer 54

Correct choice: A. Seizures Explanation: En coup de sabre means "stroke or blow of the sword" and is a term for linear morphea of the scalp/forehead. Rarely this can affect underyling structures including muscle, bone, meninges and brain and can create a focus for seizures. Sclerodactyly, Raynaud's phenomenon and cutaneous calicification are seen in systemic scleroderma or CREST syndrome, not linear morphea which is typically limited to the skin. Photosensitivity is not a feature of linear morphea.

Answer 55

Correct choice: E. Prednisone Explanation: The associated image and described biopsy findings are consistent with the diagnosis of Sweet's syndrome (aka acute febrile neutrophilic dermatosis), which typically presents with tender, erythematous edematous papules and plaques over the face and upper extremities. A vesiculobullous or pustular presentation is frequently associated with underlying myelogenous leukemia. Systemic steroids (e.g. prednisone), typically for 4-6 weeks, is the first line treatment for Sweet's syndrome barring any contraindications. Furthermore, an excellent response to systemic steroids is one of the minor diagnostic criteria for Sweet's syndrome. Potassium iodide, dapsone, and colchicine are considered 2nd line agents in the treatment of Sweet's syndrome. Therehave been rare case reports of thalidomide being effective for recalcitrant Sweet's syndrome.

Answer 56

Correct choice: B. HIV Explanation: EED has been linked to a number of systemic diseases. Infections reported to occur in association with EED have included HIV, beta-hemolytic strep, hepatitis and tuberculosis. A literature review in 2012 identified 19 reported cases of EED in association with HIV. Nodular lesions progressing to bulky masses appear to be more common in EED patients with HIV. The relationship between EED and HIV is thought to involve immune complex deposition in blood vessels triggered by the HIV infection acting as antigenic stimuli. EED also has been associated with hematologic disorders, particularly IgA monoclonal gammopathy. Additionally, EED has been linked to a variety of autoimmune diseases, such as RA, IBD and lupus.

Answer 57

Correct choice: E. Application of fluorinated topical steroids Explanation: Periorificial dermatitis (or perioral dermatitis) has been associated with prior use of topical or inhaled fluorinated steroids, therefore they should be discontinued. The other choices have not been associated with periorificial dermatitis. Doxycycline is a treatment for periorifacial dermatitis.

Answer 58

Correct choice: D. Aquagenic pruritus Explanation: Aquagenic pruritus is usually secondary to a systemic disease or another skin disorder such as urticaria or dermatographism. Primary idiopathic aquagenic pruritus is uncommon. Aquagenic pruritus presents with prickling, tingling, burning, or stinging sensations within 30 minutes of water contact, irrespective of its temperature or salinity, and lasts for up to 2 hours. Typically, symptoms begin on the lower extremities and then generalize, with sparing of the head, palms, soles, and mucosae. On exam, specific skin lesions are not seen. The pathologic mechanism is unknown although elevated dermal and epidermal level sof acetylcholine, histamine, serotonin, and prostaglandin E2 have been described. 1 - Polycythemia vera: Aquagenic pruritus can sometimes be secondary to a systemic disease such as polycythemia vera. However, the clinical scenario is a classic description of aquagenic pruritus, not polycythemia vera which presents with a ruddy complexion. 2 - Mastocytosis: Mastocytosis usually presents with cutaneous lesions and a positive Darier's sign. In patients with aquagenic pruritus, specific skin lesions are not seen. 3 - Hypereosinophilic syndrome: Cutaneous lesions are seen in > 50% of patients with hypereosinophilic syndrome. Hypereosinophilic syndrome is on the differential diagnosis of pruritus or prickling sensation provoked by water contact. In patients with aquagenic pruritus, specific skin lesions are not seen. 5 - Aquagenic urticaria: Aquagenic pruritus can sometimes be secondary to another skin disorder such as urticaria or dermatographism. However, the clinical scenario is a classic description of aquagenic pruritus, not aquagenic urticaria. In aquagenic pruritus, specific skin lesions are not seen.

Answer 59

Correct choice: C. Alginate dressing Explanation: The correct answer is alginate dressings (Choice 3) which are polysaccharides derived from kelp and algae that are ideal for highly exudative wounds. Other advantages of alginates are that they confer hemostatic benefits and are suitable for use in sinuses. Hydrocolloid dressings (Choice 1), which are waterproof gel or foam within polyurethane films, are not suitable for this patient as these dressings are good for mildly exudative wounds. Hydrogel dressings (Choice 2), hydrophilic polymer holding significant amount of water, are not suitable for this patient as these dressings are best for dry, necrotic wounds. Films (Choice 4), thin layers of elastic polyurethane, provide a barrier against bacteria and are not the best choice for highly exudative wounds. Foams (Choice 5), hydrophobic polyurethane sheets with hydrophilic surface, are ideal for mildly to moderately exudative wounds.

Answer 60

Correct choice: B. Start isotretinoin weight-based dosing after checking baseline labs and enrolling in iPledge Explanation: With a patient who has failed topical treatment who has severe acne burden with scarring, the correct choice is to start isotretinoin after enrolling in iPledge and checking baseline labs. With severe acne with scarring in a male patient who has failed topical treatment, the next most appropriate step is to initiate isotretinoin after enrolling in iPledge and checking baseline labs.

Answer 61

Correct choice: A. Lung cancer Explanation: 80% of cases of erythema gyratum repens have been associated with underlying malignancy. Lung cancer is the most common neoplasm. The skin eruption preceedes the detection of malignancy by an average of 9 months

Answer 62

Correct choice: A. Tryptase Explanation: The most common form of cutaneous mastocytosis is urticaria pigmentosa. It is diagnosed by measuring serum tryptase levels. The new diagnostic method is measurement of tryptase in bone-marrow blood, which is a new, sensitive marker of the mast cell burden in bone marrow of patients with systemic mastocytosis. The other markers do not diagnose mastocytosis.

Answer 63

Correct choice: E. Iodides Explanation: Exposure to iodides has been reported to cause flares of dermatitis herpetiformis (DH). This question assesses the examinee’s knowledge of triggers to be avoided in DH. Oral as well as topical exposure to iodides (choice 5) has been reported to cause flares of DH. Patients must strictly avoid consumption of gluten, which is found in wheat, barley, and rye; safe dietary grains include corn (choice 1), rice (choice 2), and oats (choice 3). Sulfites (choice 4) do not cause flares of DH.

Answer 64

Correct choice: A. Lisinopril Explanation: A number of drugs can cause angioedema without wheals. The most common are NSAIDs and ACE inhibitors. Aspirin intolerance may present with angioedema alone, or with urticaria or anaphylaxis; cross-reactions with other NSAIDs can occur. A causative link to ACE inhibitors may be overlooked because angioedema can present over a year after starting the medication. For practical purposes, ACE inhibitors should be discontinued in favor of other antihypertensive medications if angioedema occurs without wheals. ACE inhibitors are contraindicated in patients with HAE and acquired C1 inh deficiency. 2-Vancomycin is not the most common cause of angioedema out of the medications listed. 3-Ampicillin is not the most common cause of angioedema out of the medications listed. 4-Trimethoprim-sulfamethoxazole is not the most common cause of angioedema out of the medications listed. 5-Doxycycline is not the most common cause of angioedema out of the medications listed.

Answer 65

Correct choice: C. Erythema marginatum Explanation: Erythema marginatum: Erythema marginatum rheumaticum is a migratory, annular and polycylic erythematous eruption. It is a cutaneous manifestation of acute rheumatic fever. Associated findings incude: carditis, migratory polyarthritis, Sydenham chorea, and subcutaneous nodules. It is seen more commonly in children than in adults. 1 - Annular erythema of infancy: The differential diagnosis of erythema marginatum inclues annular erythema of infancy however the patient is not an infant in this scenario. The typical associated findings of acute rheumatic fever are absent in this condition. 2 - Erythema migrans: Erythema migrans is an annular erythema that develops at the site of a bite of a Borrelia-infected tick. Several species of Ixodes ticks are infected with different genospecies of Borrelia burgdorferi. Erythema migrans represents the initial cutanoues manifestation of Lyme disease and is seen in 60-90% of patients diagnosed with the disease. Multiple secondary lesions that are smaller in size can occur as a result of spirochetemia or lymphatic spread. 4 - Erythema gyratum repens: Erythema gyratum repens is a figurate erythema that is migratory and copmosed of concentric rings with a wood-grain appearance. It represents a paraneoplastic phenomenon and the most common underlying neoplasm is carcinoma of the lung. The lesions may have associated pruritus and scale, and they characteristically exhibit rapid migration (up to 1 cm per day). The cutaneous lesions resolve when the neoplasm is successfully treated. 5 - Erythema annulare centrifugum: Erythema annulare centrifugum is characterized by erythematous annular lesions that migrate centrifugally. Superficial lesions can have the classic trailing white scale, while the deep gyrate erythemas have a more infiltrated border. The disorder occurs more commonly in adults, and superficial lesions favor the thighs and hips. Individual lesions usually last for several days to a few months. Although often idiopathic in nature, it can be associated with infecitons (i.e. tinea pedis) and anecdotally with other disorders or exposures.

Answer 66

Correct choice: C. Arsenic Explanation: Chronic arsenic exposure can occur through contaminated drinking water or occupational exposure. It is characterized by mottled hyperpigmentation with areas of hypopigmentation, keratoses on the palms and soles and other sites, alopecia, and multiple non- melanoma skin cancers, particularly Bowen’s disease (SCCis). Arsenical keratoses are precancerous papules on the palms and soles that are symmetric, yellow and punctate. They measure 2-10 mm in size and favor the thenar and hypothenar eminences, distal palms, lateral fingers, dorsal aspect of the interphalangeal joints, and weight-bearing plantar surfaces. They rarely occur on the trunk, proximal extremities, eyelids and genitalia and may coalesce into plaques. 1 – Mercury poisoning and exposure may present with acrodynia, tattoo reaction (cinnabar), granulomas, exanthema, slate-gray discoloration, allergic and irritant contact dermatitis and baboon syndrome. 2 – Lead poisoning may present with irritant contact dermatitis and a gingival lead line. 4 – Exposure to bismuth may present with irritant contact dermatitis and black tongue. 5 – Chromium exposure may present with allergic and irritant contact dermatitis, skin ulceration, and burns. Systemic absorption can lead to renal failure, hepatic failure, anemia, and coagulopathy.

Answer 67

Correct choice: E. Pruritic, atrophic scarring of the genitalia Explanation: Antibodies to ECM-1 are associated with lichen sclerosus, choice 5. Lichen sclerosus is characterized by pruritic, atrophic plaques of the vulva, though this condition can affect the male genitals or extragenital skin as well. Choice 1 describes Lipoid Proteinosis, which is caused by a mutation in ECM-1, as opposed to antibodies. Choice 2 described Pseudoxanthoma elasticum, which is caused by a mutation in ABCC6. Choice 3 describes Pachyonychia Congenita, a family of diseases associated with mutations in keratins 6 and 16. Choice 4 describes bullous pemphigoid (or a BP-like presentation of EBA), which is caused by antibodies to BPAG1 or 2 (or Collagen 7 in the case of EBA).

Answer 68

Correct choice: A. Idiopathic Explanation: The most common causes of erythroderma are idiopathic, dermatitis, psoriasis, drug reactions, and cutaneous T-cell lymphoma. In a study of 746 patients, the underlying causes of erythroderma were dermatitis (24%), psoriasis (20%), drug reactions (19%), and CTCL (8%). When categories within the dermatitis group were examined, atopic dermatitis (9%) was the most common type, followed by contact dermatitis (6%), seborrheic dermatitis (4%), and chronic actinic dermatitis (3%). Despite multiple skin biopsies, in-depth clinical investigations, and detailed medical histories, the underlying cause of erythroderma cannot be found in 25% of patients. Cases of idiopathic erythroderma also tend to be chronic and are more likely to recur after treatment. 2 – According to a study of 746 patients, dermatitis represents 24% of the most common underlying causes of erythroderma. When categories of the dermatitis group were examined, atopic dermatitis (9%) was the most common type, followed by contact dermatitis (6%), seborrheic dermatitis (4%), and chronic actinic dermatitis (3%). 3 – Cutaneous T-cell lymphoma represents about 8% of the most common underlying causes of erythroderma. 4 – Psoriasis represents about 20% of the most common underlying causes of erythroderma. 5 – Drug reactions represent approximately 19% of the most common underlying causes of erythroderma.

Answer 69

Correct choice: D. Scabies prep Explanation: The diagnosis is scabies and therefore a scabies prep is the appropriate next step in management. Characteristic features include burrows and crusted papules in the webbing between digits. Other commonly involved areas include the areolae, umbilicus and in particular the penis and scrotum where characteristic nodules may form. In infants, the head may be involved, which is rarely seen in adult patients. A scabies prep can be performed using a #15 blade with a drop of mineral oil followed by evaluation under the microscope. A wound culture is used to evaluate for bacterial or candidal infection. Pustules should be unroofed and the purulent contents swabbed. A tzanck smear is performed to evaluate herpetic infections. The scrapings from the base of an unroofed blister reveal multinucleated giant cells. A potassium hydroxide prep is performed when there is clinical suspicion of a fungal infection. Most often, the rash will be annular with a leading scale. A punch biopsy is performed when the diagnosis is in question. A punch biopsy is selected when the suspected pathology involves the deeper skin structures. Though a biopsy could confirm the diagnosis of scabies, a scabies prep is an in office or bedside procedure offering rapid diagnosis.

Answer 70

Correct choice: E. Ventral pterygium Explanation: Lichen planus of the nails occurs in 10-15% of cases, usually in combination with other LP lesions on the skin. 20-nail dystrophy (trachyonychia) can be seen, but it is not diagnostic of LP. Thinning, longitudinal ridging, and distal splitting of the nail plate can occur (onychoschizia). Also, onycholysis, longitudinal striation (onychorrhexis), subungual hyperkeratosis, or anonychia can be seen. The classic finding is dorsal pterygium or forward growth of the eponychium with adherence of the proximal nail plate. Ventral pterygium is more classicaly seen in Scleroderma.

Answer 71

Correct choice: B. Multiple endocrine neoplasia type 2A Explanation: Multiple endocrine neoplasia type 2A: Notalgia paresthetica is a common condition in adults that is characterized by focal, intense pruritus of the upper back, especially over the medial scapular borders; this corresponds to the area innervated by the posterior rami of thoracic spinal nerves 2–6 (T2–T6). Occasionally, patients also report pain, paresthesias, or hyperesthesias. A characteristic finding on physical examination is a hyperpigmented patch due to chronic rubbing of the affected area. Dermal melanophages are evident histologically, and there is considerable overlap with macular amyloidosis.Most evidence suggests that notalgia paresthetica represents a sensory neuropathy. Degenerative changes of the spine corresponding to the affected dermatome(s) are evident in ~60% of affected patients, suggesting a pathogenic role for spinal nerve impingement. The posterior rami of T2–T6 pursue a right-angle course through the multifidus spinae muscle, potentially predisposing them to entrapment and injury. Notalgia paresthetica may also represent a sign of multiple endocrine neoplasia type 2A (Sipple syndrome), especially if the onset is during childhood or adolescence. 1 - Multiple endocrine neoplasia type 1: Notalgia paresthetica may also represent a sign of multiple endocrine neoplasia type 2A (Sipple syndrome), especially if the onset is during childhood or adolescence. It is not associated with multiple endrocine neoplasia type 1. 3 - Multiple endocrine neoplasia type 2B: Notalgia paresthetica may also represent a sign of multiple endocrine neoplasia type 2A (Sipple syndrome), especially if the onset is during childhood or adolescence. It is not associated with multiple endrocine neoplasia type 2B. 4 - Tuberous sclerosis: Notalgia paresthetica may also represent a sign of multiple endocrine neoplasia type 2A (Sipple syndrome), especially if the onset is during childhood or adolescence. It is not associated with tuberous sclerosis. 5 - Neurofibromatosis: Notalgia paresthetica may also represent a sign of multiple endocrine neoplasia type 2A (Sipple syndrome), especially if the onset is during childhood or adolescence. It is not associated with neurofibromatosis.

Answer 72

Correct choice: A. CD3+, CD4+, CD8- Explanation: Folliculotropic mycosis fungoides is a distinct variant of mycosis fungoides characterized by the presence of folliculotropic infiltrates with preferential involvement of the head and neck region. Patients may present with grouped follicular papules, acneiform lesions, indurated plaques and sometimes tumors. Skin lesions are often associated with alopecia and sometimes mucinorrhea. Infiltrated plaques in the eyebrows and concurrent alopecia are common and highly characteristic. In most cases, the neoplastic T cells have a CD3+, CD4+, CD8- phenotype as in classic mycosis fungoides. Admixture with CD30-positive blast cells is common. 2 – In most cases of folliculotropic mycosis fungoides, the neoplastic T cells have a CD3+, CD4+, CD8- phenotype as in classic mycosis fungoides. Admixture with CD30-positive blast cells is common. 3 – In most cases of folliculotropic mycosis fungoides, the neoplastic T cells have a CD3+, CD4+, CD8- phenotype as in classic mycosis fungoides. Admixture with CD30-positive blast cells is common. 4 – In most cases of folliculotropic mycosis fungoides, the neoplastic T cells have a CD3+, CD4+, CD8- phenotype as in classic mycosis fungoides. Admixture with CD30- positive blast cells is common. 5 - In most cases of folliculotropic mycosis fungoides, the neoplastic T cells have a CD3+, CD4+, CD8- phenotype as in classic mycosis fungoides. Admixture with CD30-positive blast cells is common.

Answer 73

Correct choice: A. Erythema multiforme Explanation:Erythema multiforme (EM) presents with target lesions favoring the extensor extremities. Without a history, the examinee must discover distinguishing features in the clinical photo to arrive at the correct diagnosis. On the dorsal hand, there are variable edematous urticarial papules, some of which feature three zones: a dusky center surrounded by two concentric rings of different colors. Although not all of the lesions are fully developed, based on the presence of typical targets, EM (choice 1) is the best answer. The differential diagnosis includes annular urticaria (choice 2), which displays central erythema or normal skin, never epidermal damage as evidence by duskiness, necrosis, or vesiculation; secondary syphilis (choice 3), which tends to favor the palms over the dorsa and usually has scale; fixed drug eruption (choice 4), which can be morphologically similar to EM but generally shows fewer lesions; and polymorphous light eruption (choice 5), which also presents on the sun-exposed extremities and may at times be difficult to distinguish from EM, but will not have true targets. In clinical practice, additional history and possibly biopsy will readily differentiate between these entities.

Answer 74

Correct choice: B. Pityriasis rubra pilaris Explanation: This presentation of a cutaneous eruption of palmer keratoderma, hyperkeratotic follicular papules ("nutmeg grater"), red-orange plaques and nail findings is most consistent with Type I pityriasis rubra pilaris. This clinical presentation less likely represents the other answer choices.

Answer 75

Correct choice: B. Reactive perforating collagenosis Explanation: The patient has reactive perforating collagenosis (RPC) characterized by collagen extruding through the skin. While it is not possible to discern which perforating disorder the patient has without biopsy and clinicopathologic correlation, the only perforating disorder option on the list is RPC. Neurotic excoriations is incorrect as excoriations are a secondary lesion and the patient has primary lesions (that are not excoriated). Disseminated or localized herpes simplex virus should present as vesicles, not papules with the appearance of central substance extrusion. Miliaria crystallina presents with translucent, small 1-2 mm vesicles that appear wet, which is not the appearance of the rash in the picture. Pyoderma gangrenosum is a neutrophil-rich inflammatory condition of the skin characterized by a non-healing ulcer with a red to purple appearance with an undermined border.

Answer 76

Correct choice: E. Pitting Explanation: Nail changes are a common feature of alopecia areata (AA), with an average prevalence of 30%, and can cause significant disfigurement and loss of function. Pitting and trachyonychia were by far the most common manifestations of AA, with an average prevalence of 20 and 8%, respectively. Red spotted lunulae, onycholysis, and punctate leukonychia were other reported nail findings in AA. Other etiologies, such as onychomycosis or lichen planus, may coexist with or confound the diagnosis.

Answer 77

Correct choice: A. Schnitzler's syndrome Explanation: Schnitzler’s syndrome is associated with IgM monoclonal gammopathy. This question tests the examinee’s knowledge of monoclonal gammopathies of note in dermatology. Of the answer choices, only Schnitzler’s syndrome (choice 1) is associated with IgM monoclonal gammopathy, in association with chronic urticaria, bone pain, and fevers. Sweet’s syndrome (choice 2) and subcorneal pustular dermatosis (choice 5) are associated with IgA gammopathy; scleredema (choice 3), with IgGκ; and scleromyxedema (choice 4), with IgGλ.

Answer 78

Correct choice: E. Reassurance Explanation: Seborrheic keratoses are benign lesions that do not require additional treatment. You may consider cryotherapy and/or curettage to seborrheic keratosis lesions that are bothersome for a patient but neither is necessary. Superficial radiation therapy is not appropriate treatment for a seborrheic keratosis. Excision with a 5 mm margin is an over-treatment for a seborrheic keratosis.

Answer 79

Correct choice: A. Granuloma annulare Explanation: Granuloma annulare is a benign condition characterized by annular dermal plaques. They are often pink to skin-colored. High potency topical or intralesional steroids may be used, though often observation and reassurance is the treatment of choice. Tinea corporis is a cutaneous dermatophyte with a characteristic annular appearance with an advancing edge. Erythema annulare centrifigum is a gyrate characterized by erythematous, annular patches and plaques with a trailing scale. This feature can help distinguish from tinea corporis. Subacute cutaneous lupus (SCLE) presents with annular and often scaly patches and plaques. It is most commonly seen in photodistributed areas. There is an association between SCLE and the anti-Ro antibody. Cutaneous larva migrans is a cutaneous hookworm infection acquired most commonly by walking barefoot on contaminated grounds. The classic serpiginous arrangement represents movement of the hookworm through the epidermis.

Answer 80

Correct choice: C. Waldenström macroglobulinemia Explanation: The stem describes a case of Type I cryoglobulinemia, which is typified by monoclonal IgM or IgG (no rheumatoid factor activity). Type I cryoglobulins are always linked to a B-cell lymphoproliferative disorder, that is, multiple myeloma, Waldenström macroglobulinemia, chronic lymphocytic leukemia, B-cell non-Hodgkin lymphoma, or hairy cell leukemia. Mixed cryoglobulinemias are characterized by monoclonal IgM (or IgG) with polyclonal IgG (Type II) or polyclonal IgM complexed with polyclonal IgG (Type III). Mixed cryoglobulinemias (type II or III) are associated with hepatitis C virus (HCV) infection, systemic autoimmune diseases, lymphoproliferative disorders, and other chronic infections.

Answer 81

Correct choice: A. Intralesional steroid injections Explanation: This image demonstrates exclamation point hairs of alopecia areata. Intralesional injection with low potency steroids is a first line treatment option.

Answer 82

Correct choice: B. Diarrhea Explanation: Apremilast is a phosphodiesterase 4 inhibitor which results in increased levels of cyclic AMP which in turn decreases production of TNF, IL-17, IL-23. It is dosed twice daily and is titrated up over 6 days to a final dose of 30 mg PO twice daily to minimize diarrhea. It is relatively more effective for palmoplantar psoriasis and scalp psoriasis. The most common side effects are GI upset/diarrhea in 20% of cases.

Answer 83

Correct choice: C. Stains PAS positive and diastase resistant Explanation: Choice 1 is actually apple green birefringensce on CONGO-RED staining; choice 2, there is no underlyin infection in lichen amyloid; choice 4, lichen amyloid demonstrates dermal amyloid deposition, not perivascular; choice 5 lichen amyloid is not associated with underlying malignancy (like primary systemic amyloid)

Answer 84

Correct choice: C. Hydroxycitronellal Explanation: The main allergen in Lily of the valley, which is found in perfumes, soaps, cosmetics, eye cream and aftershaves, is hydroxycitronellal (synthetic). Cinnamic acid and vanillin are present in Balsam of Peru, while atranorin and evernic acid are allergens in oak moss absolute.

Answer 85

Correct choice: D. IgA monoclonal gammopathy Explanation: The associated image and described biopsy findings are consistent with a diagnosis of pyoderma gangrenosum (PG), which most often presents as an acneiform papule or pustule that progresses into a rapidly expanding ulceration with an undermined border. ~50% of patients with PG have an underlying systemic condition, most commonly inflammatory bowel disease (ulcerative colitis more often than Crohn's disease). Other associated conditions include: arthritis (typically asymmetric, seronegative, monoarticular arthritis of the large joints), leukemia (usually myelogenous), multiple myeloma, IgA monoclonal gammopathy, polycythemia, Hepatitis C infection, HIV infection, and systemic lupus erythematosus. Celiac disease is associated with dermatitis herpetiformis. Ovarian cancer may underlie the development of dermatomyositis. Chronic pancreatitis is associated with pancreatic panniculitis. Lastly, paraneoplastic pemphigus may develop in patients with Castleman's disease.

Answer 86

Correct choice: B. Fordyce spots Explanation: Fordyce granules are sebaceous glands that are located in the vermilion zone of the lips and the oral mucosa. Historically, these sebaceous glands have been considered ectopic; however, because they are such a common clinical finding in the oral cavity, Fordyce granules should be regarded as a variation of normal anatomy. “Free” sebaceous glands (see below) can also be found on the eyelids (meibomian glands), areolae (Montgomery tubercles), labia minora, and prepuce (Tyson glands). 1- Montgomery tubercles are ectopic sebaceous glands found around the areolae. 3- Meibomian glands are ectopic sebaceous glands found on the eyelids. 4- Glands of Zeis are unilobar sebaceous glands located on the margin of the eyelid. The glands of Zeis service the eyelash. These glands produce an oily substance that is issued through the excretory ducts of the sebaceous lobule into the middle portion of the hair follicle. 5- Tyson glands are ectopic sebaceous glands found on the prepuce.

Answer 87

Correct choice: B. Selenium sulfide 1% lotion daily to affected areas Explanation: The patient has tinea versicolor and should be treated with selenium sulfide as the starting therapy. You may consider fluconazole by mouth intermittent dosing for severe, refractory cases of tinea versicolor but you should not use ongoing fluconazole therapy as treatment for the condition. Benzoyl peroxide/clindamycin is used for acne. Hydrogen peroxide solutions are used to treat seborrheic keratoses. Frequent moisturizers will not treat tinea versicolor.

Answer 88

Correct choice: E. Nevus depigmentosus Explanation: Nevus achromicus is interchangeable for nevus depigmentosus. It usually presents at birth or appear during early infancy as normal pigmentation increases. Most individuals will have a solitary lesion of nevus depigmentosus, but multiple lesions and segmental forms of nevus depigmentosus have been described. Nevus depigmentosus tends to persist lifelong, but remains unchanged after onset. The hypopigmented white spots of tuberous sclerosis are most difficult to distinguish from nevus depigmentosus, but lack of other cutaneous or systemic manifestaions exclude tubrous sclerosis. Lesions of vitiligo tend to be depigmented (melanocytopenic not melanopenic as in the question), and show a bright white coloration with Wood's lamp examination. Nevus anemicus is a distinct vascular birthmark characterized by blanching of cutaneous blood vessels, hence presenting as a "white" patch of skin that becomes unnoticeable when the surrounding skin is blanched with a glass slide ("diascopy"). Achromic nevus is an uncommon birthmark characterized by a well-defined pale patch. This is usually several centimeters in diameter, with an irregular but well-defined border. Shape and size varies. Often, smaller hypopigmented macules arise around the edges, resembling a splash of paint. Achromic nevus is also known as nevus depigmentosus and non-pigmented nevus. The name is not quite right, as thehypomelanotic patches of an achromic nevus are not completely white, unlike the areas of depigmentation in vitiligo, which are amelanotic, and completely lacking melanin. Achromic nevi are usually solitary, in contrast to tuberous sclerosis, where multiple pale patches occur and are called ash-leaf spots. Achromic nevus is usually noted at birth or early childhood, although lesions may not be apparent until mid-childhood in those with light-colored skin. The nevi remain stable over time. Achromic nevus most commonly arises on the trunk, but may also arise on the limbs and elsewhere.

Answer 89

Correct choice: B. Lungs Explanation: This is an example of lupus pernio, which is often associated with chronic sarcoidosis of the lungs. Although the patient may have involvement of other organs, pulmonary sarcoid is most often seen with lupus pernio.

Answer 90

Correct choice: A. Topical emollients at least three times per day and gentle skin care Explanation: The correct answer is gentle skin care and the use of emollients for the patient described who has winter's itch, otherwise known as asteatotic eczema. Topical anti-fungal and systemic anti-fungal medications are not indicated for this condition. You may use topical steroid in the future if not responsive to emollient and gentle skin care but you would start with a lower strength steroid than clobetasol. Unna wraps are helpful for individuals who are picking their skin but are not indicated for the patient in this vignette.

Answer 91

Correct choice: C. On histology demonstrate smooth muscle Explanation: The patient has a supernumerary nipple. Found in 1-6% of the general population. Histology shows increased smooth muscle, sebaceous glands that are opened directly onto skin surface, and mammary glands. There is no increased malignant potential but examination such as mammography or ultrasound has been recommended as with other breast tissue. Routine renal imaging is not recommended. Can be present in Incontinentia Pigmenti patients.

Answer 92

Correct choice: D. Renal impairment Explanation: Adult patients with Henoch-Schönlein purpura (HSP) are more likely than children to develop persistent renal impairment. This question tests the examinee’s ability to recognize HSP presenting as palpable purpura on the lower extremity and knowledge of how HSP complications differ in adults versus children. Compared to children, adults with HSP are at higher risk for renal impairment (choice 4), but less commonly experience abdominal pain (choice 1) or fever (choice 2). Hepatic impairment (choice 3) and cerebral arteriovenous malformations (choice 5) are not commonly recognized sequelae of HSP.

Answer 93

Correct choice: C. Anti-Mi-2 Explanation: This patient has dermatomyositis. Anti-Mi-2 antibodies in DM correlate with the presence of a shawl sign, cuticular changes, and good prognosis. Anti-Jo-1 antibodies correlate with pulmonary fibrosis, Raynaud's, and polyarthritis. Anti-SRP antibodies correlate with cardiac disease and poor prognosis. Anti-Ku antibodies correlate with sclerodermatomyositis. Anti-La antibodies correlate with Sjogren's syndrome.

Answer 94

Correct choice: E. PAPA syndrome Explanation: PAPA syndrome ( Pyogenic Arthritis, Pyoderma gangerosum and Acne) The image shows classic ulcerative pyoderma gangrenosum with undermined violaceous gray border. Re- epithelialization occurs from the margins and the ulcer heal usually with atrophic cribriform pigmented scar. Although the classic morphologic clinical presentation of pyoderma gangrenosum is an ulceration, there are several variants (bullous, pustular, and superficial granulomatous) which differ by their clinical presentation, location, and associated diseases.

Answer 95

Correct choice: B. Tuft resorption Explanation: Radiographic features of psoriatic arthritis include “sausage digits;” large eccentric erosions; pencil-in-cup deformities – erosive changes of the joint – phalangeal distal tip is pencil on an eroded cuplike joint space; tuft resorption – acroosteolysis; periostitis – inflammation of the periosteum; and sacroileitis. The other changes are seen on radiograph in osteoarthritis.

Answer 96

Correct choice: E. Cryoglobulins Explanation: In kids, pernio can be associated with cryoglobulins and cold agglutinins. It is triggered by cold and wet which results in acral violaceous color with burning and itching.

Answer 97

Correct choice: E. All of the above Explanation: All of these conditions are pro-coagulopathic and therefore predispose the patient to warfarin-induced skin necrosis.

Answer 98

Correct choice: D. Furosemide Explanation: Furosemide is the most common cause of druginduced BP. Sulfasalazine is another common offender.

Answer 99

Correct choice: D. CIAS1 Explanation: Patients with Muckle-Wells has amyloid AA fibrils. It is an autosomal dominant condition characterized by deafness, hives, and mutation in the CIAS1 gene

Answer 100

Correct choice: D. Transvaginal ultrasound Explanation: Dermatomyositis is an idiopathic inflammatory disease characterized by myositis and typical cutaneous manifestations. There is an increased incidence of malignancy in these patients which may precede, occur with, or follow the diagnosis of dermatomyositis. The most common form of malignancy in adult white women with dermatomyositis is ovarian cancer, thus a transvaginal ultrasound is the best answer as this is a common screening test for ovarian cancer. Other malignancies that have been associated with dermatomyositis include testicular cancer, gastrointestinal, lung and nasopharyngeal carcinomas. Although they are potentially reasonable options, neither of the remaining answer choices is the best answer because ovarian cancer is the most common form of malignancy in adult white women with dermatomyositis.

Answer 101

Correct choice: D. This patient likely has an underlying psychiatric disorder. Explanation: The correct answer is D- this is a factitial ulcer, which is evidenced by the irregular, sharp, geometric borders, lack of surrounding inflammation, and prior sites of picking (with secondary scarring and incomplete alopecia).

Answer 102

Correct choice: E. Hypertrichosis Explanation: Hypertrichosis is rarely reported in drug-induced pseudoporphyria.

Answer 103

Correct choice: A. Risperidone Explanation: Of all the answer choices, the second generation antipsychotic Risperidone is the best for delusions of parasitosis. The rest of the answer choices can be used to treat itchy and neurogenic pruritus in a number of conditions.

Answer 104

Correct choice: E. Histologically, parakeratosis with superficial and deep perivascular “cuffing” of lymphocytes may be seen. Explanation: The correct answer is E and describes erythema annulare centrifugum, with the characteristic “trailing scale” seen clinically. Erythema migrans does not have scale (A) and this is not the typical appearance for erythema marginatum (B), although both are annular. An azole antifungal will not help this (C) and plasma cells are not characteristic (E).

Answer 105

correct choice: E. Skin Explanation: Skin is the most affected organ in both acute and chronic GVHD.

Answer 106

Correct choice: D. Histologically, there is epidermal hyperplasia and elastic fibers may be decreased. Explanation: D is the correct answer. This is an acral fibrokeratoma. A and C describe a supernumerary digit, which is usually on the ulnar side of the 5th finger

Answer 107

Correct choice: C. Topical clobetasol Explanation: The correct answer is C. Topical steroids are typically first line treatment for lichen planus especially if not widespread.

Answer 108

Correct choice: E. topical irritants Explanation: In generalized pustular psoriasis, the infiltration of neutrophils dominates the histologic picture, while erythema and the appearance of sterile pustules dominate the clinical picture. It is an unusual manifestation of psoriasis, and triggering factors include pregnancy, rapid tapering of corticosteroids (or other systemic therapies), hypocalcemia, infections, and, in the case of the localized pattern, topical irritants. Recently, biallelic mutations in the gene encoding the IL-36 receptor antagonist (leading to increased production of IL-8 and enhanced responses to IL-1β) have been identified in a subset of patients with generalized pustular psoriasis. Generalized pustular psoriasis during pregnancy is also referred to as impetigo herpetiformis.

Answer 109

Correct choice: E. SMA Explanation: The correct answer is E. The picture shows a form of cutaneous mastocytosis and the remainder of the choices will help in identifying mast cells histologically. SMA does not stain mast cells, but a smooth muscle hamartoma is in the differential of a positive Darier sign.

Answer 110

Correct choice: B. Cheilitis glandularis Explanation: Cheilitis glandularis is demonstrated by inflammatory hyperplasia of the lower labial salivary glands that may result from chronic irritation and can arise in association with actinic cheilitis. The secretory ducts of the salivary glands become dilated and inflamed and appear as pinpoint red mucosal macules. The clinical presentation varies from slight hypertrophy of the lower lip to nodular enlargement resulting in eversion. 1 – Cheilitis granulomatosa is a rare, persistent, painless idiopathic chronic swelling of the lip. It is considered a manifestation of orofacial granulomatosis – a term describing orofacial swelling caused by non -caseating granulomatous inflammation in the absence of systemic dise ase. 3 –Melkersson-Rosenthal syndrome is a rare neurological disorder characterized by recurrent facial paralysis, swelling of the face and lips and a fissured tongue. 4 – Actinic cheilitis describes the characteristic changes that occur on the lower vermilion lip of individuals with moderate to severe photodamage. 5 – Pyostomatitis vegetans is seen in patients with inflammatory bowel disease. It is a chronic vegetate pyoderma of the labial and buccal mucosa. It may be associated with vegetative or ulcerat ive pyoderma gangrenosum and is characterized by large verrucous plaques that may be studded with pustules.

Answer 111

Correct choice: E. All of the above Explanation: These are all potential photosensitive reactions to voriconazole. These resolve on discontinuation of voriconazole. Close

Answer 112

Correct choice: E. Destruction of melanocytes Explanation: In tinea versicolor, the hypopigmented skin, there is a decreased density of melanosomes within keratinocytes, but no change in the melanocyte density. Abnormal melanosome production, decreased melanin synthesis, and a partial block in melanosome transfer to keratinocytes have all been suggested as underlying defects. Azelaic acid, a competitive inhibitor of tyrosinase produced by Malassezia furfur, may play a role in the decreased melanin synthesis. Although the differential diagnosis may include postinflammatory hypopigmentation (e.g. secondary to parapsoriasis), progressive macular hypomelanosis, and early vitiligo, the diagnosis of tinea versicolor is easily confirmed by examination of the associated scale in a potassium hydroxide preparation. 1- Azelaic acid, a competitive inhibitor of tyrosinase produced by Malassezia furfur, may play a role in the decreased melanin synthesis in tinea versicolor. 2- In tinea versicolor, the hypopigmented skin, there is a decreased density of melanosomes within keratinocytes, but no change in the melanocyte density. Abnormal melanosome production, decreased melanin synthesis, and a partial block in melanosome transfer to keratinocytes have all been suggested as underlying defects. 3- In tinea versicolor, the hypopigmented skin, there is a decreased density of melanosomes within keratinocytes, but no change in the melanocyte density. Abnormal melanosome production, decreased melanin synthesis, and a partial block in melanosome transfer to keratinocytes have all been suggested as underlying defects. 4- In tinea versicolor, the hypopigmented skin, there is a decreased density of melanosomes within keratinocytes, but no change in the melanocyte density. Abnormal melanosome production, decreased melanin synthesis, and a partial block in melanosome transfer to keratinocytes have all been suggested as underlying defects. 5-Vitiligo is a common depigmentation disorder resulting from the destruction of functional melanocytes in the affected skin. In patients with vitiligo, melanocytes are destroyed in the skin, the eyes, and possibly the ears.

Answer 113

Correct choice: D. Aquagenic pruritus Explanation: Aquagenic pruritus is usually secondary to a systemic disease or another skin disorder such as urticaria or dermatographism. Primary idiopathic aquagenic pruritus is uncommon. Aquagenic pruritus presents with prickling, tingling, burning, or stinging sensations within 30 minutes of water contact, irrespective of its temperature or salinity, and lasts for up to 2 hours. Typically, symptoms begin on the lower extremities and then generalize, with sparing of the head, palms, soles, and mucosae. On exam, specific skin lesions are not seen. The pathologic mechanism is unknown although elevated dermal and epidermal level sof acetylcholine, histamine, serotonin, and prostaglandin E2 have been described. 1 - Polycythemia vera: Aquagenic pruritus can sometimes be secondary to a systemic disease such as polycythemia vera. However, the clinical scenario is a classic description of aquagenic pruritus, not polycythemia vera which presents with a ruddy complexion. 2 - Mastocytosis: Mastocytosis usually presents with cutaneous lesions and a positive Darier's sign. In patients with aquagenic pruritus, specific skin lesions are not seen. 3 - Hypereosinophilic syndrome: Cutaneous lesions are seen in > 50% of patients with hypereosinophilic syndrome. Hypereosinophilic syndrome is on the differential diagnosis of pruritus or prickling sensation provoked by water contact. In patients with aquagenic pruritus, specific skin lesions are not seen. 5 - Aquagenic urticaria: Aquagenic pruritus can sometimes be secondary to another skin disorder such as urticaria or dermatographism. However, the clinical scenario is a classic description of aquagenic pruritus, not aquagenic urticaria. In aquagenic pruritus, specific skin lesions are not seen.

Answer 114

Correct choice: D. Rabies

Answer 115

Correct choice: C. Photoprotection Explanation: This is melasma and the best treatment option is aggressive photoprotection with hats and sunscreen. Hydroquinone may work if it is only epidermal melasma. Melasma is made worse by any irritation of the skin therefore aggressive procedures and treatments are typically not recommended.

Answer 116

Correct choice: B. Penicillamine Explanation: Penicillamine is a frequent cause of drug-induced pemphigus.

Answer 117

Correct choice: E. Dermatomyositis Explanation: This patient has the classic heliotrope rash with shawl sign (photosensitive rash) on the neck/chest, which is most consistent with dermatomyositis. There is no vesiculation, scale, erosions, lichenification or other features to indicate classic contact dermatitis, seborrheic dermatitis or eczematous dermatitis. The rash in the photo is photo- distributed and suspicious for a connective tissue disease. Scleroderma would have skin tightening rather than erythematous patches.

Answer 118

Correct choice: A. Most patients have a preceding history of the plaque-subtype of this diagnosis. Explanation: The correct answer is A (A is false); patients with pustular psoriasis uncommonly have a preceding diagnosis of plaque type psoriasis

Answer 119

Correct choice: E. Large puffy lips, facial nerve palsy, fissured tongue Explanation: granulomatous cheilitis has been reported with facial nerve palsy and fissured tongue, resulting in the condition known as Melkersson–Rosenthal syndrome.

Answer 120

Correct choice: E. All of the above Explanation: All these are factors related to increased risks of developing graft-versus-host disease.

Answer 121

Correct choice: D. Acute anterior uveitis Explanation: This is sarcoid which histologically shows naked granulomas. The most common ocular finding is acute anterior uveitis. The skin lesions are only found in 25% of patients.

Answer 122

Correct choice: A. Carbamazepine; HLA-B1502 Explanation: The correct answer is A. Han Chinese, Malaysian, and Thai populations are at a significantly higher risk of carrying the HLA-B1502 allele and developing SJS/TEN from carbamazepine (but not Japanese or Caucasians).

Answer 123

Correct choice: A. Cefaclor Explanation: Serum sickness-like reactions are most likely secondary to cefaclor.

Answer 124

Correct choice: A. high-potency steroids Explanation: The main aim of therapy for lichen sclerosus is to bring the disease under control as quickly as possible with the fewest side effects. For initial treatment (after the diagnosis is established), a potent topical corticosteroid such as clobetasol propionate 0.05% ointment is prescribed. One possible regimen is daily application of clobetasol propionate 0.05% ointment to the affected area for 3 months. This alleviates symptoms in the majority of patients, often within weeks. Subsequently, the corticosteroid is tapered down over 2 weeks and the clinical remission maintained by occasional application of a corticosteroid, using less than 30 g in any 6-month period. In some patients there is complete resolution of signs as well as symptoms, with the exception of the scarring, which is irreversible

Answer 125

Correct choice: B. Cholesterol Emboli Explanation: The image shows retiform purpura on the the distal lower extremity. Given the history of pulmonary arterial emboli along with eosinophilia and AKI, this is most consistent with cholesterol emboli (choice 2). Although the classic description of this is in a patient who underwent a catheterization, this entity can present from fragmentation of an thrombus. The showers of emboli result in cholesterol deposition in the distal extremities. Type II cryoglobulinemia is a vasculitis, not a vasculopathy, and would present with palpable purpura as opposed to retiform purpura (Type I cryoglobulinemia is a true vasculopathy). The patient has no history to suggest warfarin necrosis, and this entity is similar to calciphylacxis in that it affects more central, fatty areas of the body. Henoch Schonlein Purpura generally affects children (or adults with ESRD), and is a vasculitis which manifests with palpable purpura. There is no history of cocaine use to suggest levamisole vasculopathy.

Answer 126

Correct choice: E. Melanocytic nevi Explanation: Melanocytic nevi is not a common cause of multiple dermatofibromas. Although some dermatofibromas are thought to arise at sites of trauma or arthropod bites, their precise etiology is not known. Multiple eruptive dermatofibromas have been observed in patients with autoimmune disorders such as lupus erythematosus and atopic dermatitis and in the setting of immunosuppression (i.e. HIV infection). Clinically, dermatofibromas can be confused with cysts or melanocytic nevi, especially those with fibrosis. However, melanocytic nevi are not a common cause of multiple dermatofibromas. 1 - HIV infection: Multiple eruptive dermatofibromas have been observed in patients with autoimmune disorders such as lupus erythematosus and atopic dermatitis and in the setting of immunosuppression (i.e. HIV infection). 2 - Systemic lupus erythematosus: Multiple eruptive dermatofibromas have been observed in patients with autoimmune disorders such as lupus erythematosus and atopic dermatitis and in the setting of immunosuppression (i.e. HIV infection). 3 - Arthropod bites: Some dermatofibromas are thought to arise at sites of trauma or arthropod bites. 4 - Atopic dermatitis: Multiple eruptive dermatofibromas have been observed in patients with autoimmune disorders such as lupus erythematosus and atopic dermatitis and in the setting of immunosuppression (i.e. HIV infection).

Answer 127

Correct choice: A. Violaceous polygonal papules Explanation: Clinically, these lesions are polygonal papules which are consistent with lichen planus. On histology, as seen in the photos, lichen planus is characterized by saw-tooth rete ridges and a lichenoid infiltrate.

Answer 128

Correct choice: C. HHV 6 and 7 Explanation: Pityriasis rosea has been associated with reactivation of HHV 6 and 7.

Answer 129

Correct choice: E. Moisturizers containing humectants and occlusives Explanation: Moisturizers containing humectants (e.g. glycerin) and occlusives (e.g. petrolatum) help to repair the epidermal barrier in rosacea. Skincare recommendations for patients with rosacea include: o Wash with lukewarm water and use soap-free cleansers that are pH balanced o Cleansers are applied gently with fingertips o Use sunscreens with both UVA and UVB protection and an SPF ≥30 o Sunscreens containing the inorganic filters titanium dioxide and/or zinc oxide are usually well tolerated o Use cosmetics and sunscreens that contain protective silicones o Water-soluble facial powder containing inert green pigment helps to neutralize the perception of erythema o Moisturizers containing humectants (e.g. glycerin) and occlusives (e.g. petrolatum) help to repair the epidermal barrier o Avoid astringents, toners, and abrasive exfoliators o Avoid cosmetics that contain alcohol, menthols, camphor, witch hazel, fragrance, peppermint, and eucalyptus oil o Avoid waterproof cosmetics and heavy foundations that are difficult to remove without irritating solvents or physical scrubbing o Avoid procedures such as glycolic peels or dermabrasion

Answer 130

Correct choice: D. dermatomyositis Explanation: Additional manifestations of dermatomyositis range from poikiloderma of the scalp to centripetal flagellate erythema to erosions and ulcerations. It is also important to look for dermatologic signs of other connective tissue diseases in patients with dermatomyositis, because of the frequency of overlap syndromes. The most common cutaneous findings would be signs of overlap with systemic sclerosis, especially limited disease with CREST features (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), annular lesions suggestive of subacute cutaneous lupus erythematosus, or nodules suggestive of rheumatoid arthritis. Cutaneous small vessel vasculitis can occur as an associated finding, especially in patients with juvenile dermatomyositis.

Answer 131

Correct choice: A. To do an EGD Explanation: A patient with Bazex syndrome also known as Acrokeratosis Paraneoplastica should have an EGD. Patients can also have nail dystrophy , acquired keratoderma, and the skin findings usually precede the underlying malignancy. The other listed testing is not required for Bazex syndrome.

Answer 132

Correct choice: D. It usually occurs after a respiratory tract infectioon Explanation: This patient has IgA vasculitis, also known as Henoch-Schonlein purpura (HSP). HSP typically occurs after a respiratory tract infection in children. HSP is much less common in adults. A DIF biopsy showing perivascular IgA deposition is diagnostic (C3 and fibrin may also deposit perivascularly). Two percent of patients develop permanent renal impairment. Treatment is mainly supportive as HSP is typically self-limited. Corticosteroids may be effective in rapid resolution of renal manifestations and treating joint and abdominal pain, but they are not proven effective for treating organ manifestations and complications, such as glomerulonephritis, bowel infarction or intussusception. Mycophenolate mofetil or cyclosporine A may be better treatment choices in case of renal involvement. Other immunosuppressive and immunomodulating drugs, such as rituximab and dapsone, are promising, but larger studies are needed to confirm these findings. Cancer screening should be considered in older males diagnosed with Henoch-Schönlein purpura.

Answer 133

Correct choice: A. MDA5 Explanation: This patient's findings of ragged cuticles (Samitz sign), periungual erythema, and dorsal finger erosions are highly suggestive of the subtype of dermatomyositis (DM) with positive MDA5 (melanoma differentiation-associated gene 5) autoantibodies. This subtype is usually amyopathic or hypomyopathic, explaining this patient's lack of muscle weakness. It is also associated with rapidly-progressive interstitial lung disease, which accounts for her pulmonary symptoms. Mi-2 autoantibodies are associated with classic manifestations of DM (heliotrope rash, Gottron papules) and a positive prognosis. SRP autoantibodies are associated with DM with cardiac involvement and poor prognosis. TIF1-gamma autoantibodies are associated with paraneoplastic DM. Lastly, the DM anti-synthetase syndrome often has Jo-1 autoantibodies and is characterized by pulmonary disease, arthritis, Raynaud's, and myositis.

Answer 134

Correct choice: C. Apremilast Explanation: The small-molecule phosphodiesterase 4 inhibitor apremilast modulates cytokines that are up-regulated in Behçet's syndrome. In patients with oral ulcers associated with Behçet's syndrome, apremilast resulted in a greater reduction in the number of oral ulcers than placebo but was associated with adverse events, including diarrhea, nausea, and headache. Consequently, in July 2019, apremilast was FDA-approved for the treatment of oral ulcers associated with Behcet's syndrome. The remaining answers are not FDA-approved to treat oral ulcers associated with Behcet's syndrome.

Answer 135

Correct choice: C. Minocycline Explanation: The picture shows confluent and reticulated papillomatosis. There are coalescing and hyperkeratotic papules located on the central trunk. Minocycline is the most effective treatment; however, recurrence is common.Insulin may be needed for patients with acanthosis nigricans, a cutaneous marker of insulin resistance and metabolic disorders. Selenium suflide is the treatment of choice for tinea veriscolor. Tinea veriscolor is characterized by hypo or hyperpigmented macules and papules with fine scale. The diagnosis can be confirmed with a potassium hydroxide prep and visualization of clustered yeast and hyphae.Topical corticosteroids are ineffective for the treatment of confluent and reticulated papillomatosis. Isotretinoin has been reported to be effective in the treatment of confluent and reticulated papillomatosis; however, in light of the high likelihood of recurrence and side effect profile, it is not a first line treatment.

Answer 136

Correct choice: E. Anti-histone Explanation: This patient is presenting with signs and symptoms concerning for anti-phospholipid antibody syndrome (APS). Anti-histone antibodies are typically only present in the setting of drug- induced systemic lupus erythematosus (SLE), which is extremely unlikely given that this patient's only medication is albuterol. Common culprit medications for drug-induced SLE are procainamide, hydralazine, isoniazid, and minocycline. Patients with APS commonly have the remaining listed positive antibodies. APS is an autoimmune disease characterized by antiphospholipid antibodies (aPL) associated with thrombosis and/or pregnancy morbidity. Most APS events are directly related to thrombotic events, which may affect small, medium or large vessels. Other clinical features like thrombocytopenia, nephropathy, cardiac valve disease, cognitive dysfunction and skin ulcers (called non-criteria manifestations) add significant morbidity to this syndrome and represent clinical situations that are challenging. APS was initially described in patients with systemic lupus erythematosus (SLE) but it can occur in patients without any other autoimmune disease. Despite the autoimmune nature of this syndrome, APS treatment is still based on anticoagulation and antiplatelet therapy.

Answer 137

Correct choice: D. Calciphylaxis Explanation: The correct answer is E. Xrays of the affected limb may demonstrate vascular calcification within the skin; however, this may also be seen in healthy patients with renal disease that are not affected by calciphylaxis. Bone scintigraphy using technetium Tc 99m bisphosphonates in patients with calciphylaxis shows increased radiotracer uptake in soft tissues throughout the body and is specifically enhanced in indurated plaques affected by calciphylaxis (but is absent in ulcers due to reduced blood flow at sites of tissue necrosis). Calciphylaxis is a condition characterised by necrosis (cellular death) of the skin and fatty tissue. It is seen mainly in patients with end-stage kidney disease. It is also sometimes called calcific uraemic arteriolopathy or calcific vasculopathy. In 1981, approximately 50 cases of calciphylaxis were reported in the world literature. Today, the incidence is estimated at 1 per cent per year in patients undergoing renal dialysis. The mortality is extremely high, up to 80%, often within several months of onset. The primary cause of death is from secondary infection of the ulcers, and sepsis. Calciphylaxis is also called calcific uraemic arteriolopathy or calcific vasculopathy. A deep wedge skin biopsy may be necessary to diagnose calciphylaxis, as a similar appearance can be seen in other conditions such as necrotizing fasciitis, Cryoglobulinemia, antiphospholipid syndrome, coumarin necrosis, and vasculitis. Multiple biopsies may be necessary, with a risk of propagating calciphylaxis. The pathologist looks for calcium deposited within scarred and blocked blood vessels in the subcutaneous tissue. Perieccrine calcium deposition may be noted when vascular calcification is absent but may be subtle. There may also be inflammation of the fat (panniculitis).

Answer 138

Correct choice: E. The palmoplantar subtype of this diagnosis has the greatest malignant potential of all subtypes. Explanation: The correct answer is A (A is false). Linear porokeratosis has the greatest risk of malignant (SCC) transformation of all subtypes of porokeratosis. This photo depicted is disseminated superficial actinic porokeratosis (DSAP) which is seen on the legs>arms and is very difficult to treat. E describes the cornoid lamella characteristically seen in porokeratosis. The plaque (Mibelli) subtype of this diagnosis typically appears in childhood. The palmoplantar subtype of this diagnosis does not have the greatest malignant potential of all subtypes.

Answer 139

Correct choice: D. Tooth extraction Explanation: The image is of a dental sinus tract, an intraoral connection with a periapical abscess. Extraction of the nonviable tooth is essential to treatment. Topical antibiotics are not an effective treatment for dental abscesses. Oral antibitoics and incision and drainage may be needed in addition to tooth extraction for infection eradication. An excision would not address the primary problem, which is a chronic dental infection.

Answer 140

Correct choice: C. Dimethyl fumarate Explanation: Dimethyl fumarate, the contact allergen of 2011, is present in sachets to prevent the mold formation on shipped furniture. Thiuram is typically present in rubber, latex, and adhesives. Colophony or rosin is found in adhesive tape, cosmetics, insulating tape, glossy paper, polish, paints, inks, epilation wax, varnishes, violin bows, and chewing gum. Chromate is found in leather, cements, and some green felts (pool tables). Quaternium-15 is the most common cosmetic preservative to cause ACD. It is a preservative that releases formaldehyde.

Answer 141

Correct choice: D. Seborrheic dermatitis Explanation: This man has erythroderma. Seborrheic dermatitis is a rare cause of erythroderma in young children, and would be an extremely unlikely cause in an adult. Pre-existing dermatoses (primarily atopic dermatitis or psoriasis), medications, cutaneous T-cell lymphoma (especially the leukemic form of mycosis fungoides called Sezary syndrome), and pityriasis rubra pilaris are more common causes of erythroderma than seborrheic dermatitis in adults.

Answer 142

►E Schnitzler's syndrome presents as episodes of urticarial vasculitis that occur in association with a monoclonal IgM M component. Fever, lymphadenopathy, hepatosplenomegaly, bone pain, and sensorimotor neuropathy also occur.

Answer 143

►B TH1 cells produce IL-2, IFN-gamma, and TNF-beta, and are associated with cell-mediated immunity. TH2 cells produce IL-4, IL-5, IL-6, IL-10, and IL-13, and are associated with antibody- mediated immune responses.

Answer 144

►C Black tongue is associated with poor oral hygiene, the use of medications, and radiation to the head and neck region. In many cases, simply brushing the tongue with a toothbrush or using a commercially available tongue scraper is sufficient improve the condition.

Answer 145

►E The image shown is inverse type of psoriasis. Although psoriatic lesions are classically distributed on the extensor surfaces (the elbows, knees, and lumbosacral regions), lesions may also be found in a flexural distribution with involvement of the axillae, groin, perineum, central chest, and umbilical region. Differential diagnosis include all other choices in question. Although erythrasma might be included in differentials in case of groin involvement only, it is unlikly for eryhrasma to presnt on penis with well demarctaed erythamtos plaques. Erythrasma is a superficial bacterial infection of the skin caused by C. minutissimum. It is characterized by asymptomatic, well-demarcated, reddish brown, slightly scaly patches in the groin, axillae, gluteal crease, or inframammary regions, and less often the interdigital spaces of the feet. Erythrasma is frequently confused with a dermatophyte infection, it can be differentiated from tinea infection by the characteristic coral red fluorescence seen when viewed under Wood's lamp illumination (due to the production of porphyrins by the corynebacteria.

Answer 146

►C Desogestril, norgestimate and gestodone are progestins with low intrinsic androgenic properties.

Answer 147

►A Patients with acne fulminans can have severe acne lesions with lytic lesions indicative of sterile osteomyelitis seen on x-ray and bone scans. The sternoclavicular joint and the chest wall are most frequently affected. Treatment includes oral prednisone, antibiotics and isotretinoin.

Answer 148

►C Rifampin is a potent hepatic microenzyme inducer and has been shown to reduce efficacy of oral contraceptives. Anecdotal reports of decreased contraceptive efficacy on oral antibiotics have not been confirmed in the literature. The putative mechanism is decreased absorption of hormones secondary to altered gut microflora.

Answer 149

►D Secondary systemic amyloidosis presents with deposition of amyloid in the adrenals, liver, spleen, and kidney as a result of some chronic disease, such as TB, leprosy, Hodgkin's, Behcet's, rheumatoid arthritis, ulcerative colitis, schistosomiasis, or syphilis. The skin is not involved. AA amyloid fibrils, derived from SAA protein (an acute phase reactant) are deposited. AA is also seen in Muckle-Wells and familial mediterranean fever. Biopsy of normal skin may be positive for perivascular amyloid. Dialysis-related amyloidosis occurs via deposition of beta 2microglobulin component altered by uremia, and resuls in carpal tunnel syndrome, bone cysts, and spondyloarthropathy.

Answer 150

►B This condition is vitamin B12 deficiency and is characterized by glossitis and hyperpigmentation in sun exposed areas and creases. Neurologic abnormaolities and megaloblastic anemia can be seen. The symptoms can mimic folic acid deficiency. Riboflavin (B2) is associated with oralocular- genital syndrome. Carcinoid tumors as well as azathioprine, 5-FU, and isoniazid are associated with niacin deficiency. Eating raw eggs is a risk factor for biotin deficiency.

Answer 151

►C HLA-B57, HLA-Cw6, or HLA-DR7 are most commonly associated with early onset type I psoriasis. The presence of HLA-B13 or B17 is associated with a 5-fold risk of developing psoriasis and are increase in guttate and erythrodermic psoriasis. HLA-B27 may be seen in pustular psoriasis. HLA-Cw2 is seen with late onset psoriasis, or type II. HLA-DR3 is commonly found with subacute cutaneous lupus

Answer 152

►D Topical antibiotics reduce the population of P. acnes on the skin, and thus are indirectly anti inflammatory. In contrast to topical retinoids, topical antibiotics are not comedolytic. They are generally well tolerated by patients.

Answer 153

►B Multicentric reticulohistiocytosis is a non-Langerhans histiocytosis associated with mutilating arthritis. Dermatologically, patient may present with coral beading around the fingers. There is a 30% incidence of malignancy in these patients.

Answer 154

►D Fabry's disease is inherited is x-linked recessive. Female carriers often exhibit mild forms of this disorder. It can be establish that a patient has FD by searching for low activity of alpha galactosyl A in plasma, leukocytes, cultured skin fibroblasts, or dried blood spots on filter paper. Because of the Lyon effect, enzymatic detection of carriers can be misleading; thus, specific genetic analysis can be helpful in making the diagnosis.

Answer 155

►A Familial Mediterranean Fever is described above. It is autosomal recessive with incomplete penetrance. The main therapy is Colchicine. PAPA syndrome, TNF receptor associated periodic syndrome, hyper-IgD syndrome and familial cold autoinflammatory syndrome are related autoinflammatory syndromes. PAPA syndrome is Pyogenic Arthritis, Pyoderma gangrenosum and Acne and is caused by proline serine threonine phosphatase-interacting protein (PSTPIP1) or CD2- binding protein 1 (CD2BP1) which interact with pyrin. TNF receptor associated periodic syndrome (TRAPS) has similar findings to FMF, but the attacks are of longer duration, is autosomal dominant in transmission, and does not respond to colchicine. TRAPS is caused by a mutation in the TNFRSF1A gene which results in a decrease in soluble TNF receptor. Hyper IgD syndrome is associated with defects in the mevalonate kinase gene, which presents with a hereditary periodic fever. Familial cold autoinflammatory syndrome is associated with cryopyrin defects and is characterized by fever, rash, conjunctivitis and arthralgia upon exposure to cold.

Answer 156

►C Acne vulgaris is a disease of follicular hyperkeratosis and the microcomedone is thought to be the precursor lesion. P. acnes has lipase that cleaves triglycerides into free fatty acids. P. acnes can activate complement and PMN chemotaxis. Toll-like receptors (TLR) recognize bacterial patterns and P. acnes activates TLR-2.

Answer 157

►A The cells secreting the glucagon tumor are the alpha islet cells of the pancreas. This patient has necrolytic migratory erythema which is associated with pancreatic glucagonoma, malignancies, liver disease, malabsorption states, and inflammatory bowel disease.

Answer 158

►C There are three autosomal recessive syndromes that can lead to biotin deficiency: holocarboxylase synthetase deficiency, biotinidase deficiency, and an inability to transport biotin into cells. All of these syndromes present with a dermatitis similar to that of zinc deficiency as well as neurologic abnormalities

Answer 159

►E This slide shows comedonal acne. Topical retinoids are the treatment of choice. Salicylic acid containing cleansers may be helpful adjuncts to treatment with mild comedolytic properties.

Answer 160

►A Anti-Jo-1 antibodies against histidyl-tRNA synthetase are described in patients with dermatomyositis and pulmonary disease. Anti-Mi-2 antibodies against nuclear helicase are described in classic DM with a good prognosis. Patients with Wegener's disease display cANCA antibodies against proteinase-3 in a cytoplasmic pattern. Antiphospholipid antibodies are also known as anticardiolipin antibodies and lupus anticoagulant. They can cause a false positive VDRL. Clinical features include livedo reticularis, thrombotic events and spontaneous abortions. Anti- histone antibodies are seen in drug-induced lupus.

Answer 161

►A Natural measles infection has been shown to ameliorate atopic dermatitis.

Answer 162

►D The FDA classifies medication safety for use in pregnancy as follows: Category A: Controlled studies in humans show no risk to fetus. Category B: Controlled human studies show no risk (but may show risk to animals), or no risk in animal studies (but no human studies have been conducted). Category C: Risk to human fetus cannot be ruled out, studies are lacking; animal studies are equivocal. Category D: Controlled studies show risk, but in some instances benefits may outweigh risks. Category X: Contraindicated in pregnancy. The question describes category B. Azeleic acid is a category B medication. Benzoyl peroxide, topical tretinoin, and TMP/SMX are category C medications. Tetracycline is a category D medication.

Answer 163

►B Kveim-Siltzback test is for sarcoidosis, Montenegro-leishmanin test is for Leishmaniasis, and lepromin test or histamine/methacholine sweat test can be used for leprosy. Kveim -Siltzback test is done by intradermal injection of a suspension from granuloma-containing spleen, lymph node, or other tissue from a confirmed case of sarcoidosis. A positive test is characterized by the formation of a papule at the site of injection within 4-6 weeks which, on microscopic examination, exhibits non-necrotizing granulomas and the absence of foreign material. This teast is rarely done nowdays because of the difficulties involved in preparation, standardization and validation of the test material as well as significant variation in the sensitivity and specificity of test suspensions obtained from different sources.

Answer 164

►A A patient with a persistent mongolian spot with indistinct borders, persistent or progressive behavior may have an underlying lysosomal storage disease, most commonly GM1 gangliosidosis type I and Hurler disease.

Answer 165

►A A patient with Bazex syndrome also known as Acrokeratosis Paraneoplastica should have an age appropriate cancer screening. Patients can also have nail dystrophy , acquired keratoderma, and the skin findings usually precede the underlying malignancy.

Answer 166

►C This condition is called Sweet's syndrome, or acute febrile neutrophilic dermatosis. Diagnosis relies on two major and two minor criteria. Major ones include 1) an abrupt onset of juicy painful plaques and bullae and 2) neutrophilic infiltration in the dermis on pathology. Minor criteria include 1) presence of associated conditions ie inflammatory bowel disease, infections, pregnancy, leukemia, etc 2) fever and malaise, 3) laboratory values ie high ESR and CRP, and 4) excellent response to prednisone.

Answer 167

►D Reiter‘s syndrome is a chronic inflammatory disease similar to psoriatic arthritis. Commonly, patients present with either a peripheral, asymmetric arthritis, or a urethritis, or conjunctivitis. Not all of these findings need be present. Patients are usually men with the HLA -B27 genotype. Other clinical findings include fever, weight loss, keratitis, iritis, and cardiac disease. Skin lesions are often found on the palms (keratoderma blenorrhagicum _ hyperkeratotic papules and plaques) or the penis (balanitis circinata). Reiter‘s follows an infection of either the genitourinary tract or the gastrointestinal tract.

Answer 168

►E Camptodactyly is a flexion contracture of the 3rd through the 5th proximal interphalangeal joints and elbows bilaterally. Blau syndrome is an autosomal dominant form of sarcodosis due to a defect in the CARD15 gene manifesting also with arthritis, cutaneous sarcoid, uveitis, and synovial cysts. It does not have lung or visceral involvement.

Answer 169

►A Type I (familial lipoprotein lipase deficiency or apoprotein CII deficiency) hyperlipoproteinemia has increased chylomicrons as a feature, and presents with eruptive xanthomas and lipemia retinalis. For the other hyperlipoproteinemias, the associated lipid abnormalities are as follows: Type IIa -- increased LDL; Type IIb -- increased LDL and VLDL; Type III -- increased IDL; Type IV -- increased VLDL. Type V has increased chylomicrons and increased VLDL.

Answer 170

►E Local lidocaine can be safely used but systemic lidocaine can precipitate anaphylaxis in patients with mastocytosis. Other systemic analgesics that are contraindicated in mastocytosis include succinylcholine, d-tubocurarine, thiopental, metocurine, enflurane, isoflurane, and etomidate. Propofol, vecuronium bromide, and fentanyl are safe options for systemic anesthesia in patients with mastocytosis.

Answer 171

►D This patient has primary systemic amyloidosis. Mucocutaneous findings can be seen in up to 40% of patients. The surface of the tongue may be smooth and dry or covered with waxy papules and nodules. Teeth indentations may be seen along the lateral borders. The most common cutaneous signs are petechiae and purpura that occur spontaneous or after minor trauma ("pinch purpura" about the eyelids). The most characteristic skin lesions are waxy, shiny, smooth papules and nodules which are usually hemorrhagic or flesh-colored. Flexural areas are common sites. These patients may also present with carpal tunnel syndrome, hepatomegaly, and edema.

Answer 172

►A Ascher syndrome is a rare syndrome characterized by recurrent upper (and sometimes lower) eyelid swelling as well as upper vermillion and cutaneous lip swelling. The latter often yields the appearance of a ―double upper lip,ǁ while multiple episodes of the former results in stretching and redundancy of the eyelid skin, causing blepharochalasis. Ascher syndrome is frequently seen in association with a euthryoid (non-toxic) goiter, although up to 50% of patients may have no associated thyroid abnormality. The pathogenesis of this condition remains unknown.

Answer 173

►A Graham-Little-Piccardi-Lassuer syndrome has a triad of follicular LP of the skin and or scalp, multifocal cicatricial alopecia of the scalp and non-scarring alopecia of axillary and pubic areas.

Answer 174

►A Pitted keratolysis is a skin disorder characterized by crateriform pitting that primarily affects the pressure-bearing aspects of the plantar surface of the feet. It is caused by a cutaneous infection with micrococcus sedentarius which is included in the Corynebacteria genus. These are grampositive, catalase-positive, aerobic or facultatively anaerobic, generally nonmotile rods. Corynebacterium diphtheriae infection is classically characterized by a local inflammation, usually in the upper respiratory tract, associated with toxin-mediated cardiac and neural disease. Corynebacterium tenuis causes trichomycosis Patients typically present with yellow, black, or red pinpoint nodules on the hair shafts in the inguinal region. Staphylococcus aureus is a common bacterium that can result in formation of pustules, furuncles, cellulitis and abscesses. Candida species are responsible for superficial infections such as oropharyngeal candidiasis (thrush) and vulvovaginal candidiasis (vaginal Candidiasis) and is also occurs as an opportunistic infection

Answer 175

►B It is most likely a granulomatous inflammatory disease with infiltrates of macrophages arranged around focus of degenerated collagen and mucin. The etiology is unknown: metabolic disturbances, autoimmune, allergy, and infection.

Answer 176

►E Patients with paraneoplastic pemphigus has painful oral lesions and polymorphous skin eruption. The patients die from complications of underlying malignancy. Bronchiolitis obliterans has also been reported as a complication and cause of death.

Answer 177

►D Scleredema is a type of dermal degenerating mucinosis characterized by diffuse symmetric induration of the upper body. 3 types of scleredema have been described. The first type is seen in children following a stretococcal infection. The second type is associated with a monoclonal gammopathy. The third type is related to insulin dependent diabetes.

Answer 178

►A Reiter's syndrome is associated with HLA-B27 and can have a variable course often 3-12 months. There is a chronic sequelae in 15-20% of patients.

Answer 179

►A Patients diagnosed with Grover's disease have exacerbation commonly in the winter time. Authors suggest that there may be due to xerotic epidermis and impaired epidermal integrity and decreased sweat production. It is not caused by sweating and heat and is not increased in summer.

Answer 180

►A Sneddon's syndrome presents as livedo reticularis and livedoid vasculitis associated with ischemic cerebrovascular lesions, hypertension, and extracerebral arterial and venous thromboses.

Answer 181

►B Nodular amyloidosis is a rare type of primary cutaneous amyloidosis made up of AL protein. The risk for systemic involvement is 7%.

Answer 182

►A ANA-negative SLE is uncommon. The ANA has a high negative predictive value and low positive predictive value. ANA-negative SLE can result if a patient only makes antibodies to ssDNA, which is not detected by most tests.

Answer 183

►A Type XVII collagen, or BPag2 (180 kD) has been associated with LP pemphigoides. The hemisdesomosome plauqe is also known as BPag1 (230 - kD), desmoglein 1 and 3 are associated with the Pemphigous family, and antibodies to Type VII collagen is found in Epidermolysis Bullosa Aquisita

Answer 184

►A First line therapy for subcorneal pustular dermatosis or Sneddon-Wilkinson disease is dapsone 50-200mg/day. Sulfapyridine, acitretin, PUVA, NB UVB, topical and systemic steroids, vitamin E and antibiotics have been reported to be helpful also.

Answer 185

►E In a large retrospective study of 1383 patients, diabetes mellitus was diagnosed in 21% of patients with generalized GA, compared with 9.7% of patients with localized GA. Othere choices also have been reported to be associated with granuloma annulare.

Answer 186

►A Angiolymphoid hyperplasia with eosinophilia often presents with red to reddish-brown papules or nodules on the head or neck. While lesions can occur on any body site, most common locations are the ears and scalp.

Answer 187

►B Xanthoma striata palmaris are diagnostic of type III dysbetalipoproteinemia (AR; broad beta disease). This condition also presents with palmar, planar, tendinous, tuberous, eruptive, and intertriginous xanthomas, increased IDL, and atherosclerosis. It is associated with diabetes, gout, and obesity.

Answer 188

►C Relapsing polychondritis is a chronic, recurrent rheumatic disease characterized by cartilaginous inflammation involving the nose, ears, and trachea. A non-erosive inflammatory arthritis may also be present, and affected patients may suffer from neurosensory hearing loss, tinnitus, and/or vertigo secondary to vestibular or cochlear damage. Patients may have circulating autoantibodies to type II collagen (found exclusively in cartilage) with circulating titers corresponding to disease activity.

Answer 189

►E Acne and acneiform eruptions can be caused or exacerbated by cortiosteroids, oral contraceptives, androgens, ACTH, lithium, phenytoin, halogens, INH, and haloperidol.

Answer 190

►B Doxepin is the treatment of choice for patients with neurotic excoriations. It has both antidepressant and antipruritic effects. Combined psychiatric and pharmacologic intervention is recommended. Other agents such as risperidone, olanzapine, gabapentin, and diphenhydramine are not drugs of choice for this disorder.

Answer 191

►A Niacin (Vitamin B3) deficiency is associated with a photosensitive dermatitis, diarrhea and dementia. The photosensitive dermatitis classically involves the face, neck and upper chest (Casal's necklace) and forearms (as pictured). Other findings may include angular cheilitis and thickening and hyperpigmentation of skin overlying bony prominences.

Answer 192

►A Lichen sclerosis is most commonly located in the genitalia. Longstanding lichen sclerosis is thought to be a risk factor for the development of a squamous cell carcinoma.

Answer 193

►B Sub acute cutaneous lupus presents with scaly papules that evolve into either psoriasiform or polycyclic annular lesions (more commonly). Sun-exposed surfaces of the face and neck are commonly involves. Photo sensitivity is seen in 40%, the hard palate is involved in 40%, and concomitant DLE is seen in 20%.

Answer 194

►B This is the most common digital manifestation in patients with SLE and it could present in up to 30% of the patients. It is a paroxysmal vasospam of digits in response to cold exposure or emotional stress.

Answer 195

►B Sarcoidosis is a systemic disease charcterized by non-caseating granulomas. Organ involved include the skin, lung, liver and eyes. Lupus pernio manifests as indurated, red-brown, swollen plaques of the nose, lips, cheeks and ears.

Answer 196

►E Keloids are dense overgrowths of fibrous tissue that generally form a response to tissue injury. Lobomycosis and dermatofibromasarcoma protuberans may mimic keloids.

Answer 197

►D It is one of several vascular lesions that can present on the skin during pregnancy. Other common vascular lesions include palmar erythema, spider angiomas, varicose veins, cavernous hemangiomas, glomus tumors, and hemorrhoids. Etiologic factors in development of vascular lesions during pregnancy thought to be a combination of increased hormones and increased intravascular pressure. Pyogenic granulomas may develop as a reaction to trauma. They often regress after delivery and treatment is not usually required unless they cause excessive bleeding.

Answer 198

►D Nekam's Disease (keratosis lichenoides chronica) presents with violaceous papules and nodules, hyperpigmented and hyperkeratotic, covered with gray scales. There is often a linear and reticulate pattern on the dorsal hands and feet, extremities and buttocks. This condition is generally very refractory to treatment.

Answer 199

►D Hereditary angioedema is an autosomal dominant condition associated with recurrent attacks of angioedema of the skin, respiratory, and gastrointestinal tract, without urticaria and is caused by a functional deficiency in C1 esterase inhibitor. In contrast, acquired angioedema is caused by a depletion in C1 esterase inhibitor. There are two forms of acquired angioedema; one results from the production of antibodies directed against C1 esterase inhibitor, and the other is seen in association with underlying malignancies, especially B cell lymphomas, with antibody production against overexpressed paraproteins. Acquired angioedema can be distinguished from hereditary angioedema by differences in serum levels of C1. While the level is markedly decreased in acquired angioedema, a normal C1 is seen in patients with hereditary angioedema.

Answer 200

►A Gali-gali syndrome is a variant of Dowling Degos syndrome. It is characteristic of 1- to 2-mm slightly keratotic red to dark brown papules which are focally confluent in a reticulate pattern. The disease is also characterized by slowly progressive and disfiguring reticulate hyperpigmentation of the flexures.

Answer 201

►B Acquired C1 esterase inhibitor deficiency occurs in the setting of lymphoproliferative disease, monoclonal gammopathy of undetermined significance, or rheumatologic disease and results in a decreased serum C1q level. Inherited C1 esterase inhibitor deficiency shows an autosomal dominant inheritance, and earlier presentation, and a normal level of serum C1q. In the inherited disease, the C1 esterase inhibitor may display normal levels but be functionally impaired.

Answer 202

►A Esophageal dysfunction is the most systemic finding in systemic sclerosis (>90%). Dysphagia results from decreased peristalsis and may occur before skin findings are seen. Small intestinal involvement is also common. Pulmonary fibrosis, myocardial fibrosis (seen in 50-70%), cardiac conduction defects, heart failure, pericarditis with effusion, and renal disease with slowly progressive uremia may all be seen.

Answer 203

►D Acne fulminans may be associated with lytic bone changes which are indicative of a sterile osteomyelitis. The sternoclavicular and chest wall joints are most frequently affected.

Answer 204

►A A person that always burns and sometimes tans is type II skin.

Answer 205

►D Dermatomyositis is an autoimmune polymyositis with characteristic cutaneous findings. Patients with dermatomyositis should be screened for a underlying malignancy. Risk factors for having an underlying malignancy include a negative ANA, adult age, and female gender. Ovarian cancer is one of the most frequently associated with dermatomyositis.

Answer 206

►D This patient has psoriatic nail changes and a history suggestive of psoriatic arthritis. Psoriatic arthritis is an inflammatory arthropathy associated with psoriasis. In 80% of patients the rheumatoid factor is negative; however a positive rheumatoid factor may sometimes be seen. 80% of patients present with skin disease first. Large eccentric erosions are classically present on radiographs. Cyclosporine is not effective in treating psoriatic arthritis. Mild skin disease may be associated with moderate-to-severe joint disease, and vice versa.

Answer 207

►B A low serum alkaline phosphatase, a zinc dependent enzyme, may be a valuable adjuctive test where the serum zinc level is normal or near normal. The diagnosis of zinc deficiency should be suspected in at-risk individuals with acral or periorificial dermatitis. It is usually confirmed by low serum zinc levels.

Answer 208

►C Sjogren's syndrome is a triad of keratoconjunctivitis sicca, xerostomia, and rheumatoid arthritis. More than 90% of patients are women. Labial salivary gland biopsy is useful for diagnosis, and the Schirmer test for xerostomia detects diminished glandular secretions. Laboratory findings often include positive cryoglobulins, anti-Ro, anti-La, and RF positivity. Antibodies to fodrin are 93% specific for this diagnosis.

Answer 209

►D Annular elastolytic giant cell granuloma is characterized by asymptomatic annular plaques on sun exposed surfaces. Elastic fibers are absent in the center of lesions. The disease is seen primarily in middle aged women.

Answer 210

►C The picture shows classical case of erythema nodosum (EN). Although all other choices in question cause EN, the most common in the pediatric patient is beta-hemolytic streptococcal infection (especially pharyngitis). The management of erythema nodosum is directed at identification and treatment of the underlying cause. Minimal evaluation usually involves obtaining an ASO/DNase B titer, chest radiograph, and tuberculin testing. Bed rest, with elevation of the patient's legs, helps reduce pain and edema. When pain, inflammation, or arthralgia is prominent, NSAIDs can be prescribed. Salicylates, colchicine, and potassium iodide are the most commonly used alternative therapies.

Answer 211

►D Relapsing polychondritis involves intermittent episodes of inflammation of the articular and nonarticular cartilage, resulting in chondrolysis, dystrophy, and atrophy of the cartilage. Both sexes are equally affected. IgG anti-type II collagen antibodies are pathogenic, with titers corresponding to disease activity found in up to 50% of patients with relapsing polychondritis (and in only 15% of those with RA). Involvement is often unilateral. Migratory arthralgias are seen in 50-80%. The course is unpredictable, often chronic and variable with episodic flares. Relapsing polychondritis causes death in 1/3 of patients secondary to airway collapse, cardiovascular complications, and infection (secondary to systemic steroids).

Answer 212

►D Alcoholism is the main cause of nutritional disease in developed nations. Other conditions that cause nutritional disease include: unusual diets, postoperative state, psychiatric illness, inflammatory bowel disease, cystic fibrosis, surgical bowel dysfunction, and inborn errors of metabolism.

Answer 213

►E This patient has Vogt-Koyanagi-Haradi syndrome (VKHS). VKHS is marked by bilateral granulomatous uveitis, vitiligo, deafness, poliosis, and alopecia. This syndrome begins with a meningoencephalitic phase with fever, malaise, headaches, nausea, and vomiting. Varying degrees of neurological impairment may be present. Recovery is usual complete. The second phase is the ophthalmic-auditory phase during which uveitis appears rapidly and can results in blindness. Treatment with systemic steroids may prevent blindness. Lisch nodules are iris hamartomas which are seen in neurofibromatosis. Corneal opacities are seen in X-linked ichthyosis. Retinal detachment can occur as a result of head trauma, as in shaken-baby syndrome.

Answer 214

►A Glomus tumors appear as one of the painful tumors that are reddish spots on the nail.

Answer 215

►A Patients that have discoid lupus erythematosus have a 5% chance of developing systemic lupus erythematosus. DLE is complicated by scarring and there is also a risk for development of squamous cell carcinoma and basal cell carcinoma.

Answer 216

►C Ethyl cyanoacrylate is a chemical found in Krazy Glue. It can also be found in nail adhesives causing a dermatitis on the face and fingers, nail dystrophy, and a generalized rash. Formaldehyde is a widely used chemical found in a variety of applications and is a common allergen. It can be found in paper, skin and hair products, cosmetics, and permanent press textiles. Lanolin alcohol is found in wool fat, wool wax, adhesives, cosmetics and pharmaceuticals. Octyl-dimethyl-paba is a chemical found in sunscreens and is the most frequently used PABA group sunscreen. Mercaptobenzothiazole is a rubber accelerant and is the most common allergen found in dermatitis to shoes. It is also found in veterinarian products such as flea and tick sprays and powders. Mercapotbenzothiazole can also be found in cutting oil, antifreeze, fungicides and photographic film emulsions.

Answer 217

►A Muckle Wells is an autosomal dominant disorder with periodic attacks of urticaria, fever, deafness, and renal amyloidosis. The implicated protein is AA, which is derived from SAA, an acute phase reactant.

Answer 218

►A This obese patient has acanthosis nigricans and acrochordons, both of which may be associated with insulin resistance and diabetes mellitus. While she may very well have hypertensio n, hyperlipidemia, hypercholesterolemia, and peripheral vascular disease, these are not significantly related to her cutaneous findings. Acanthosis nigricans may also be seen in patients with gastric adenocarcinoma.

Answer 219

►A The emotional and physical burden of psoriasis is easil y underestimated. Rapp et al found that patients with psoriasis report reduced physical and mental functioning (―heath related quality of lifeǁ) similar to that seen in patients with cancer, hypertension, arthritis, heart disease, diabetes, and depression.

Answer 220

►A The patient described likely has dialysis associated amyloidosis. Beta 2-microglobulin is the protein component which is involved, and can become deposited in various tissues causing, among other thing, carpal tunnel syndrome, bone cysts, and spondyloarthropathy.

Answer 221

►D On biopsy, alopecia areata exhibits a peribulbar lymphocytic ―swarm of bees.ǁ Discoid lupus typically exhibits inflammation surrounding the isthmus, along with a perivascular dermatitis and vacuolar interface changes. Lichen planopilaris exhibits inflammation most densely concentrated about the infundibulum.

Answer 222

►C Orf, caused by the parapox virus is usually contracted by direct exposure to infected sheep or goats. Milkerâ€™s nodules are caused by a closely related virus found in cows. Both of these viruses can be contracted by exposure to fomites (fence post, soil) containing the virus. One to several lesions may develop usually on the hands or forearm and generally resolve without therapy in 4-6 weeks.

Answer 223

►C The condition shown is balnitis circinata which is part of the constellation of findings in Reiter's syndrome in addition to arthritis, urethritis, and conjunctivitis. There is a higher incidence of this condition people with HLA-B27.

Answer 224

►A Morphea is a inflammatory disease primarily of the dermis and subcutaneous fat that ultimately leads to a scar like sclerosis. Clinically, morphea can be divided into plaque, linear, and generalized. There is some thought that Borrelia infection may play a role in mor phea. mRNA specific for Borrelia has been found in some lesions of morphea. This association however is controversial.

Answer 225

►B Amoxicillin is in the penicillin family of antibiotics and is an immunologic cause of urticaria. Immunologic urticaria is most commonly caused by exposure to this family and other related beta- lactam antibiotics. Patients with reaction to penicillins have an increased risk of crossreacting to cephalosporins, mostly the earlier generations. The third-generation cephalosporins are less likely to cause reactions in a penicillin allergic patient. The other listed options are causes of non- immunologic urticaria. They alter prostaglandin metabolism which increases degranulation of mast cells.

Answer 226

►C Pre-existing dermatoses is most common cause in non-HIV patients, including atopic dermatitis, psoriasis, seborrheic dermatitis, chronic actinic dermatitis, mycosis fungoides, pityriasis rubra pilaris, and allergic contact dermatitis. Drugs are the second most common overall, and the most common in HIV patients

Answer 227

►E Becker's nevus can be occasionally associated with smooth muscle hamartoma, hypoplasia of underlying structures, such as unilateral breast hypoplasia, unilateral or ipsilateral pectoralis major aplasia, ipsilateral limb shortening, ipsilateral foot enlargement, spina bifida, scoliosis, pectus carinatum, localized lipoatrophy, congenital adrenal hyperplasia, polythelia, and accessory scrotum. In addition to acneform lesions and eczematous dermatitis. There is no association with cardiac defect.

Answer 228

►A Insulin resistance is the most common cause of acanthosis nigricans. Insulin-like growth factors, produced by the liver in response to high levels of circulating insulin, bind epidermal growth factor receptors to produce thickening of the epidermis and hyperkeratosis. Cushing's syndrome, acromegaly and polycystic ovarian syndrome are associated with acanthosis nigricans, although less common than insulin resistance. Paraneoplastic acanthosis nigricans is associated with gastrointestinal carcinoma, classically gastric carcinoma.

Answer 229

►E The antinuclear antibody (ANA) pattern most specific for CREST is the anti-centromere pattern. The specificity rate is approximately 50-90% and carries a more favorable prognosis than Scl-70. The target protein for the anti-centromere pattern is the kinetochore.

Answer 230

►D HLA-Cw6 is the HLA type most definitively associated with psoriasis. It carries a relative risk 915 times normal.

Answer 231

►C Primary cutaneous amyloidosis presents as either macular or lichen amyloidosis. The protein component is keratin. Macular amyloidosis often presents over the upper back, while lichen amyloid presents over the shins.

Answer 232

►C This patient has Frey syndrome or auriculotemporal nerve syndrome. This is characterized by facial flushing, sweating, or both localized to the distribution of the auriculotemporal nerve that occurs in response to gustatory stimuli. In adults, the syndrome usually results from surgical injury or trauma to the parotid gland.

Answer 233

►D Tosylonomide formaldehyde resin is found nail polish and is a common cause of eyelid dermatitis in women.

Answer 234

►A Conidia function as asexual organs. Favic chandeliers, pectinate bodies, racket forms and mycelium have no reproductive capabilities.

Answer 235

►A Acrokeratosis paraneoplastica, also known as Bazex syndrome, is a rare paraneoplastic syndrome. Clinically, it appears as symmetric, hyperkeratotic lesions on red base, in an acral distribution, nose and helices of the ears. Most commonly associated with upper aerodigestive tract tumors. Treatment of the tumor leads to disappearance of lesions.

Answer 236

Answer A Centrofacial psoriasis may be associated with more severe body psoriasis. Palmoplantar psoriasis can be difficult to treat. Patients that have psoriatic arthritis can be candidates for biologic therapy.

Answer 237

►C Acne fulminans is a rare, explosive form of severe cystic acne affecting young males. Patients may be systemically ill, with leukocytosis, fever, arthralgias, and myalgias. Lytic changes, indicative of a sterile osteomyelitis, can be seen on x-ray and bone scans. The sternoclavicular joint and chest wall are most frequently affected. Treatment is with oral prednisone, intralesional steroids, antibiotics, and isotretinoin.

Answer 238

►A Bazex's syndrome (acrokeratosis paraneoplastica) is a paraneoplastic dermatosis associated with malignancy of the aerodigestive tract. Bazex's syndrome classically presents with violaceous erythema involving the ears, nose, hands and feet. Lesions progress to become hyperkeratotic and psoriasiform in appearance. The other answer choices all represent paraneoplastic dermatoses, but none are as closely associated with aerodigestive tract malignancies as Bazex's syndrome.

Answer 239

►E In a recent case series and review (Arch Dermatol. 2010; 146 (5): 513-516) it was shown that the following are associated with post-transplant CTCL: renal transplant, cyclosporine, tacrolimus, and male sex. There have been 29 cases of post-transplant CTCL documented in the literature to date.

Answer 240

►A Reiter syndrome, now referred to as reactive arthritis (ReA), is a condition that most often occurs following enteric or urogenital infections. Reactive arthritis is associated with human leukocyte antigen (HLA) B27, although HLA-B27 is not always present in individuals who are HIV+. Bacteria associated with reactive arthritis are generally enteric or venereal and include the following: Shigella flexneri, Salmonella typhimurium, Salmonella enteritidis, Streptococcus viridans, Mycoplasma pneumonia, Cyclospora, Chlamydia trachomatis, Yersinia enterocolitica, and Yersinia pseudotuberculosis.

Answer 241

►B Isotretinoin is a synthetic retinoid that is used primarily in the treatment of acne. Isotretinoin has a half-life of approximately 20 hours. Women should be advised to avoid becoming pregnant for at least one month.

Answer 242

►B Human-Beta-Defensin (HBD) and cathelicidins are innate antimicrobial peptides in human skin. HBD-1 is constitutively expressed. HBD-2 (and the cathelicidin LL 37) is expressed in response to skin injury and inflammation.

Answer 243

►C Initial screening test in angioedema for both inherited and acquired should include C4, which is low. C3 is normal in angioedema. C1q is low in acquired angioedema but normal in the hereditary type. Bradykinin is elevated in both inherited and acquired angioedema.

Answer 244

►D The picture shows generalized type of vitiligo or vitiligo universalis. Patients who have widespread disease with only a few areas of normally pigmented skin in exposed sites can be treated with depigmenting agents. The patients must be carefully chosen, i.e. adults who recognize that their appearance will be altered significantly and who understand that depigmentation requires lifelong care of the skin (sunscreens, protective clothing, etc.). The most commonly used agent is monobenzyl ether of hydroquinone (MBEH) 20% applied twice daily to the affected areas for 9-12 months or longer. Monobenzyl ether of hydroquinone is a potent irritant and/or allergen, and an open use test should be performed before more widespread application. It normally takes 1-3 months to initiate a response, and a loss of pigment can occur at distant sites. Although depigmentation from MBEH is considered permanent, repigmentation (especially perifollicular) can be seen following a sunburn or even intense sun exposure. Monomethyl ether of hydroquinone in a 20% cream can be used as an alternative to MBEH. Side effects include contact dermatitis, exogenous ochronosis and leukomelanoderma en confetti. Phototherapy and excimer laser are not good or practical choices for this type of vitiligo. Nitrogen mustard is not used in vitiligo.

Answer 245

►D Carcinoid tumors are derived from enterochromaffin (Kulchitsky) cells. The appendix is the most common site, followed by the ileum. Less than 4% of abdominal carcinoid tum ors have the carcinoid syndrome; the presence of the syndrome implies hepatic metastases, extraabdominal carcinoid tumor, or a large enough tumor burden such that the liver cannot degrade the increased level of hormone. Foregut tumors (bronchus, stomach, pancreas) produce histamine, cause peptide ulcers, and produce more persistent, intense flushing, and lacrimation, sweating, vomiting, and asthma. Midgut tumors (small-intestine to mid-colon) cause cyanotic flush, hypotension, and bronchoconstriction more commonly. Hindgut tumors (descending colon and rectal) do not lead to flushing or other manifestations.

Answer 246

►B Carcinoid syndrome involves tumors derived from the enterochromaffin cells. The appendix is the most common site. Tumors can be foregut (bronchus, stomach, or pancreas), midgut (small intestine to mid-colon), or hindgut (descending colon and rectal). Tumors from the foregut area produce histamine, cause peptic ulcers, and produce intense, persistent flushing and lacrimation.

Answer 247

►C Balanitis circinata presents as sharply demarcated, serpiginous ulcers or plaques on the penile head. Balanitis circinata is usually seen in Reactive arthritis which has a classic triad of arthritis, urethritis, and conjunctivitis. The syndrome typically occurs post-infection of the GI or urinary tract.

Answer 248

►E Neonatal LE presents with annular scaling erythematous macules and plaques on the head and extremities within the first few months of life in babies born to mothers with LE, rheumatic diseases, or other connective tissue disorders. 50% of mothers are asymptomatic at delivery. Lesions resolve spontaneously by 6 months, healing without scarring. Photosensitivity may be prominent. 75% of cases involve girls. 50% have congenital heart block, which is permanent, and may be the only manifestation of the disease. Thrombocytopenia and hepatic disease are as frequent as cardiac disease.

Answer 249

►B Linear nigra is one the latest skin changes in pregnancy. It is a hyperpigmented, linear streak which extends from the pubic symphysis to the xiphoid process. It usually appears from t he 20th week.

Answer 250

►B Keratin is the protein component in primary cutaneous amyloidosis. No amyloid is found around blood vessels. Macular amyloidosis (which may have associated notalgia paresthetica) and lichen amyloidosis are forms of primary cutaneous amyloidosis. Secondary cutaneous amyloidosis presents with keratin-derived amyloid and is seen following PUVA therapy and in benign and malignant neoplasms.

Answer 251

►D Juvenile xanthogranulomas are the most common form of non-Langerhans cell histiocytoses. 80% of these lesions appear within the first year of life and they present as firm, red-brown to yellowish papule. The most common noncutaneous site is the iris, so referral to ophthalmologist is warranted.

Answer 252

►B The patient has Behcet's disease. Behcet's disease is diagnosed based on recurrent oral ulceration (at least 3 times in a 12 month period) plus 2 of the following: recurrent genital ulceration, posterior uveitis, skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions, or acneiform nodules), and a positive pathergy test. Behcet's disease is associated with HLA-B51.

Answer 253

►C C-kit staining is specific for acral melanocytic lesions. Ras is found in all melanocytic lesions. P53 mutations are found in actinic keratoses, SCCIS, and SCC.

Answer 254

►C Gram negative folliculitis may occur after prolonged systemic antibiotic use for acne vulgaris. It should be suspected in patients who develop a sudden acneiform eruption after having been stable for some time. The treatment of choice is isotretinoin.

Answer 255

►A The most common associated malignant neoplasm is basal cell carcinoma in a nevus sebaceous of Jadassohn.

Answer 256

►A Twenty-nail dystrophy, nail plate splitting and pterygium formation are nail changes associated with Lichen Planus. Darier's disease is associated with longitudinal red and white streaks of the nail plate and V-shaped knicking distally. Psoriasis is associated with many nail abnormalities including pitting, onycholysis and oil-spots. Scleroderma may cause nail fold capillary dilation and destruction while patients with dermatomyositis may exhibit nail fold telangiectasias and frayed cuticles.

Answer 257

►A Inherited Quincke's edema have a normal C1q levels. Type I has low amounts of normal C1 sterase inhibitors and type II has normal amounts of dysfunctional C1 esterase inhibitor.

Answer 258

►A Internal hordeolum are secondary infection of the Meibomian gland in the tarsal plate. The infectious agent is Staphylococcus aureus in 90-95% of cases.

Answer 259

►A Nifedipine is effective in about 70% of patients with pernio in prevention of the development of new skin lesions. The other options listed, other than aspirin, are anecdotally suggested to be helpful.

Answer 260

►A This patient has a heliotrope rash and proximal muscle weakness, consistent with the diagnosis of dermatomyositis. 18-32% of patients with dermatomyositis will develop malignancy. The risk of malignancy is greatest within the first 3 years of diagnosis. Ovarian cancer is overrepresented. In Southeast Asia, nasopharyngeal cancer is overrepresented.

Answer 261

►B Spironolactone is a potassium-sparing aldactone antagonist. Renal failure predisposes patients to potentially dangerous hyperkalemia.

Answer 262

►B Probiotics have been studied in the primary prevention of atopic dermatitis by Kalliomaki et al. Lactobacillus GG cultures were given to pregnant women with a history of atopy to assess the effect of potentially beneficial gut flora on the prevention of atopic disease in their children. The frequency of atopic dermatitis in the children in the probiotic group was half that in the placebo group at two years of life.

Answer 263

►D The most common laboratory abnormality seen in patients taking isotretinoin is increased triglycerides, followed by elevation of ALT and AST.

Answer 264

►E The question describes a case of pityriasis rosea, which has been associated with HHV6,7. There is an increased incidence in immunocompromised patients. There is an increased risk of miscarriage in women who develop pityriasis rosea before 15 weeks of gestation. Acyclovir may be effective in the treatment of pityriasis rosea, especially if treated in the first week of presentation (JAAD 2006;54(1):82-5).

Answer 265

►C An abnormal paraproteinemia is found in approximately 90% of cases with scleromyxedema, generally IgG lambda. IgA paraproteinemia can be seen in pyoderma gangrenosum and Sneddon- Wilkinson disease. Amyloidosis and NXG can be associated with IgG kappa paraproteinemia, and Schnitzler syndrome is associated with an IgM paraproteinemia.

Answer 266

►B Twin concordance (identical twins) is 55% in alopecia areata. There is a high frequency of positive family history, especially in patients with early onset (37%). The inner root sheath and matrix are immune privileged, not expressing MHC class I, and this privilege may collapse in alopecia areata. The sisapho or ophiasis inversus pattern of alopecia areata is a bandlike pattern of the fronto parietotemporal scalp. Atopic dermatitis is a predictor of poor prognosis in patients with alopecia areata.

Answer 267

►E Most patients with calciphylaxis have chronic renal insufficiency and hyperparathyroidism. The associated risk factors are diabetes mellitus, obestiy, dialysis, warfarin therapy, iron and vitamin D replacement, weight loss, and liver disease.

Answer 268

►A Lichen Amyloidosis is a primary cutaneous amyloidosis. Deposits of amyloid of believed to be derived from necrotic keratinocytes. AL deposits are seen in systemic amyloidosis which is usually associated with a monoclonal gammopathy or myeloma and the amyloid is derived from immunoglobulin light chains. Amyloid deposits composed of AA are seen in secondary systemic amyloidosis such as familial Mediterranean fever and Muckle Wells syndrome, amyloid deposits composed of transthyretin are seen in familial amyloidotic polyneuropathy and amyloid deposits composed of Beta2-microglobulin are seen in hemodialysis patients

Answer 269

►B Henoch-Schonlein Purpura occurs mostly in children. There is an antecedent URI in 75% of cases. HSP involves the skin, synovia, GI tract, and kidneys. Long-term morbidity results from renal disease, which is predicted by the spread of purpura to the upper trunk. Skin lesions of adults show blisters and necrosis.

Answer 270

►D Immunologic abnormalities in atopic dermatitis include increased synthesis of IgE, increased specific IgE to multiple allergens, increased expression of CD23 (low-affinity IgE receptor) on B cells and monocytes, increased basophil histamine release, impaired DTH response, decreased CD8 suppressor/cytotoxic T-cell number and function, increased secretion of IL-4 and IL-5 by TH2 cells, and decreased secretion of IFN-gamma by TH1 cells.

Answer 271

►A Reiter's syndrome is characteristics of urethritis, conjunctivitis, and arthritis. It occurs in young men of HLA-B27 genotype that rarely occurs in women.

Answer 272

►C Sarcoidosis presenting with fever, cough, joint pains, hilar adenopathy and erythema nodosum is known as Lofgren's syndrome. Erythema nodosum is the most common nonspecific cutaneous finding in sarcoidosis. Lofgren's syndrome occurs frequently in Scandinavian whites and is uncommon in American blacks.

Answer 273

►C Sweet's syndrome is a dramatic skin disease characterized by eruptions of tender, pseudovesicular coalescing papules and plaques most commonly appearing on the face, neck and upper trunk. It is seen in association with fever, leukocytosis and typically responds promptly to systemic steroid therapy. Cutaneous pathergy has been described. The etiology of Sweet's syndrome is unclear. A hypersensitivity reaction to a bacterial, viral or tumor antigen has been postulated. Nearly 20% of cases are seen in association with an underlying malignancy, particularly hematologic malignancies, with acute myelogenous leukemia being the most common. In addition, drug-related variants of Sweet's syndrome have been described. The following drugs have been implicated: granulocyte colony-stimulating factor (G-CSF), all-trans retinoic acid, hydralazine, carbamazepine, levonorgestrel/ethinyl estradiol, trimethoprim/sulfamethoxazole, and minocycline.

Answer 274

►A Patients with erythema gyratum repens have a "wood grain appearance" and is most commonly associated with lung carcinoma. Other cancers associated with erythema gyratum repens is breast, cervical, bowel, and bladder cancer.

Answer 275

►D Lichen planus-related nail changes seen here include thinning of the nail plate with onychorrhexis, ridging and pterygium formation.

Answer 276

►A Patients with albinism have a normal number of epidermal melanocytes, however, these melanocytes synthesize inadequate amounts of melanin. Vitiligo, piebaldism, Waardenburg's syndrome and Ziprowski-Margolis syndrome all feature a decreased number or total absence of epidermal melanocytes.

Answer 277

►E Linear morphea is the most common presentation in children, comprising between 40% to 70% of children with morphea. This subtype includes linear morphea of the extremity, en coup de sabre, or progressive facial hemiatrophy, all of which may be accompanied by underlying tissue atrophy.

Answer 278

►E Lichen planus pemphigoides presents with tense blisters atop lesions of LP or de novo on uninvolved skin. It can be differentiated from bullous LP, where blisters develop in lesions of longstanding LP as a result of intense lichenoid inflammation and extensive liquefactive degeneration of basal keratinocytes. Histologically it resembles LP, with linear deposition of IgG and C3 at the DE junction. Circulating IgG autoantibodies react to a 180/200 kDa antigen within the basement membrane zone.

Answer 279

►E Extracutaneous manifestations of Sweet's syndrome occur in more than 75 percent of patients with Sweetǁs syndrome. The most common is fever, followed by arthralgias, arthritis, or myalgias. Other less frequent manifestations include conjunctivitis, episcleritis, oral aphthaelike lesions, cough, dyspnea, pleuritis, and sterile multifocal osteomyelitis. Cardiac, renal, hepatic, intestinal, or neurologic manifestations are rare.

Answer 280

►A Phrynoderma (toad skin) presents clinically as keratotic papules on the extremities and shoulders and results from a deficiency of Vitamin A. Vitamin A is a fat soluble vitamin. Hypovitaminosis A is often seen in diseases such as Crohn's disease, celiac disease and cystic fibrosis which manifest with fat malabsorbtion. Hypovitaminosis A is additionally associated with night blindness, xeropthalmia and keratomalacia.

Answer 281

►C Retinoid activity in humans is mediated by retinoid receptors. Two groups exist: RA receptors (RAR) and RX receptors (RXR). Each has three receptor subtypes: alpha, beta, and gamma. RAR- gamma is the most important mediator of retinoid activity in the skin.

Answer 282

►B Both isotretinoin and tetracycline are known to cause pseudotumor cerebri. In combination, the risk is significantly elevated. Symptoms of pseudotumor cerebri include headaches, pulsatile tinnitus, diplopia, and blurred vision.

Answer 283

►C Familial mediterranean fever and Muckle-Wells (renal amyloidosis, urticaria, fevers, limb pains, and deafness) involve AA. MENA IIa involves keratin-derived amyloid. Familial amyloidotic polyneuropathy (FAP) type III involves mutations in apolipoprotein A-1; FAP type IV involves mutations in gelsolin.

Answer 284

►C Nevus anemicus is caused by localized hypersensitivity to catecholamines and most commonly found in the upper chest

Answer 285

►A Dermatofibrosarcoma protuberans is typically located on the trunk. The extremities are the second most common location for this type of neoplasm.

Answer 286

►E Anthralin, in addition to possessing antiproliferative activity on human keratinocytes, has strong anti-inflammatory effects by inhibiting PMNs and monocytes. Vitamin D3 analogues are inactivated by salicylic acid and should be used after UV light (calcipotriol absorbs UV). Retinoids reduce scaling and plaque thickness, but do not generally decrease lesional erythema. Anthralin can stain hair purple and cause reversible brownish discoloration of surrounding skin.

Answer 287

►A This woman has scleroderma which is a systemic disease characterized by skin induration and thickening. The cutaneous findings are accompanied by various degrees of tissue fibrosis and chronic inflammatory infiltration in numerous visceral organs, prominent fibroproliferative vasculopathy, and humoral and cellular immune alterations. Antinuclear antibodies are present in about 95% of the patients, usually with a speckled or homogenous pattern. A nucleolar pattern, although less common, is more specific for systemic sclerosis. Topoisomerase I antibodies (formerly Scl-70) are present in approximately 30% of patients with diffuse disease (absent in limited disease) and are associated with pulmonary fibrosis. Anticentromere antibodies are present in about 60-90% of patients with limited disease and are rare in patients with diffuse disease. Fibrillarin antibodies and antibodies to U3 ribonucleoprotein (RNP) may also be present but are more common in patients with skeletal muscle involvement. Anti-U3RNP is present mostly in patients with diffuse disease with overlap syndromes. Anti-ThRNP is present mostly in limited disease and is associated with more extensive visceral disease. Anti-PM-Scl is present in limited and overlap states and is associated with myositis and renal involvement.

Answer 288

►C C1 esterase inhibitor is a protease inhibitor that inhibits the catalytic subunits of the first components of the classical pathway. In the absence of C1 esterase inhibitor, activated C1 and plasmin generate activated C2 kinin, which mediates angioedema. Acquired C1 esterase inhibitor deficiency generally affects adults or elderly individuals with no family history. Serum C1q is decreased. It occurs in the setting of lymphoproliferative disease or rheumatologic illness, where idiotype/anti-idiotype immune complexes consume available C1q and functionally and quantitatively lower the amounts of C1 esterase inhibitor. It can also occur in the setting of autoimmunity directed against the C1 esterase protein. Inherited C1 esterase inhibitor deficiency is detected in the first or second decade of life and is autosomal dominantly inherited. Serum C1q is normal in the inherited form, but there is a defect in the synthesis and/or function of C1 esterase inhibitor. In both the inherited and acquired forms levels of C2 and C4 are decreased because of the uncontrolled actions of C1s.

Answer 289

►C Idiopathic hirsutism is diagnosed in women with evidence of androgen excess but with normal menses, normal-sized ovaries, no evidence of tumors of the adrenal or ovary, and normal androgen function. Slight elevations of plasma androstenedione and testosterone are common.

Answer 290

►B Discoid lupus erythematosus (DLE) presents with plaques characterized by scarring, atrophy, follicular plugging, and scale and photosensitivity. Children presenting with DLE have a higher incident of developing systemic lupus (SLE) than adults. Because of progression from DLE to SLE, children should be screened and followed with antinuclear antibodies and anti - DNA antibodies. Children and adolescents have a higher incidence of renal involvement. Treatment for DLE includes topical steroids, oral steroids, and hydroxychloroquine.

Answer 291

►C The patient is likely experiencing erythema nodosum (EN), a delayed hypersensitivity response to a wide variety of eliciting factors. They consist of an eruption of erythematous, tender nodules, typically over the anterior tibial areas. Common causes include oral contraceptives, which is a probable choice given that the patient is likely taking them as part of her isotretinoin treatment. Strep infection is also a common cause of EN but with no mention of symptoms is less likely in this case. Other causes include TB, fungal infections, sarcoidosis, ulcerative colitis, and regional enteritis.

Answer 292

►C The patient depicted has dermatomyositis. Autoantibodies to anti-Jo-1 antibody targets histidyl transfer RNA synthetase. In dermatomyositis correlates with the development of pulmonary disease.

Answer 293

►A The most common eye complication of sarcoidosis is anterior uveitis with mutton fat keratotic precipitates. All the others can be a complication of sarcoidosis also.

Answer 294

►D Psoriatic nail changes may be of nail matrix or nail bed origin. Pits are the common finding; splinter hemorrhages the least. Psoriatic nail changes of matrix origin include: pits (representing focal psoriasis of the proximal matrix) and leukonychia. Psoriatic nails changes of nail bed origin include: salmon spots, oil spots, onycholysis, subungual hyperkeratosis, and splinter hemorrhages.

Answer 295

►B This patient has acrodermatitis enteropathica, a rare, inhertied disorder caused by an inability to absorb zinc. This disease is characterized by a traid of acral dermatitis, diarrhea, and alopecia. AE is rapidly reversed by zinc supplementation. Alkaline phosphatase is a zinc-dependent enzyme; it is a moderately-sensitive marker for zinc deficiency (although not an early marker).

Answer 296

►A Lichen amyloidosis and Macular amyloidosis are derived from degenerated tonofilaments of keratinocytes. Primary systemic amyloidosis results from deposition of protein AL derived from Ig Lambda light chains. Nodular amyloidosis is also associated with AL type protein. Secondary systemic amyloidosis is associated with AA amyloid fibrils derived from SAA protein. Dialysis related amyloidosis is associated with beta-2-microglobulin protein deposition.

Answer 297

►E PRP can affect any portion of the body, but commonly affect the extensor surfaces of the extremities as well as the sides of the neck and trunk. It may progress with an almost erythrodermic type state with characteristic small island of normal skin within affected areas. Phrynoderma refers to the toadskin like skin seen in some vitamin deficiencies including vitamin A deficiency.

Answer 298

►A Desmoplastic trichoepithelioma is referred to as sclerosing epithelial hamartoma. The location is usually on the face of women

Answer 299

►C Azithromycin is a macrolide antibiotic. It binds the bacterial 50s ribosomal subunit and inhibits RNA-dependent protein synthesis.

Answer 300

►C Gram negative folliculitis may occur after prolonged antibiotic therapy for acne vulgaris. It should be suspected in patients who are well controlled and then suddenly flare. The treatment of choice is isotretinoin.

Answer 301

►C Behcet's disease is characterized by recurrent oral ulceration plus 2 of the following: recurrent genital ulceration, eye lesions (posterior uveitis), skin lesions, positive pathergy test. Clinical features include thrombosis of the superior vena cava, thrombophlebitis, CNS lesions that give a picture of multiple sclerosis, and an asymmetric non-erosive polyarthritis.

Answer 302

►A Seborrheic dermatitis is characterized by greasy, yellow, scaly plaques involving the scalp, central face, and chest. It can be treated with medications such as ketoconazole or selenium sulfide. Psoriasis is defined by well defined round, scaly, pink plaques on the knees and elbows. Seborrheic keratoses are defined by stuck on waxy papules and plaques. Rosacea may be characterized by pink papules and telangiectasias on the cheeks. Dermatomyositis is characterized by heliotrope violaceous periocular patches.

Answer 303

►A A number of studies have indicated an increased risk of non-Hodgkins lymphoma and enteropathy T-cell lymphoma in patients with dermatitis herpetiformis. There is also increased prevalence of thyroid disease, type I diabetes, and other autoimmune disorders, such as vitiligo, Addison's, and alopecia areata.

Answer 304

►B Reiter's syndrome is a chronic inflammatory disease similar to psoriasis with psoriatoc arthritis. Reiter's syndrome usually occurs in men of HLA-B27 genotype and rarely occurs in women. Few patients present with the classic triad, thus can be diagnosed with peripheral arthritis >1 month and associated urethritis. Keratoderma blennorrhagicum is crusted, hyperkeratotic papules and plaques on plantar surfaces. Painful and bloody urination and pyuria, cystitis, prostatitis and seminal vesiculitis may occur secondary to Chlamydia trachomatis. Nail lesions as described may occur.

Answer 305

►A Sarcoidosis is a non-infectious granulomatous disorder of unclear etiology. Ocular manifestation are relatively common in sarcoidosis and may include acute anterior uveitis (classic finding), posterior uveitis, lacrimal gland enlargement, and conjuctival nodules.

Answer 306

►A This patient has Mikulicz's syndrome which is sarcoid of the parotid, submandibular and lacrimal glands. Heerfordt-Waldenstromn syndrome demonstrates a combination of fever, parotid enlargement, anterior uveitis, and facial nerve palsy. Darier-Roussy sarcoid is characterized by subcutaneous nodular sarcoid on the trunk and extremities. Lofgren's syndrome features acute sarcoid, erythema nodosum and migratory polyarthritis, fever, iritis, and bilatera l hilar adenopathy. Blau's syndrome is a rare autosomal dominant familial granulomatous syndrome that presents with arthritis, uveitis, and skin lesions that appear as "red dots;" there is no pulmonary involvement.

Answer 307

►D Several antibodies have prognostic importance in dermatomyositis. Anti-Mi is associated with an improved prognosis. Anti-Jo is associated with a poor prognosis and lung involvement. SRP is also associated with a poor prognosis and heart involvement.

Answer 308

►B Erythema Nodosum (EN) is represented by tender, warm,nodules and plaques, often but not exclusivley located on the anterior shin. It represents a reactive panniculitis. Causes include, infections (Streptococcal, tuberculosis, yersina, mycoplasma, campylobacter,salmonella, histoplasmosis, blastomycosis, coccidiomycosis), drugs (sulfonamides, gold, and OCP's), enteropathies, pregnancy, hodgkin's disease and lymphoma and sarcoidosis. Loefgren's disease is a varient of sarcoidosis with EN, hilar adenopathy, fever, uveitis and arthritis. Differential diagnosis includes: pretibial myxedema and erythema induratum.

Answer 309

►D Whorled corneal opacities are classically seen in Fabry's disease. Pseudo-herpetic ulcerations are seen in Richner-Hanhart. Brushfield spots can be seen in Down's syndrome. Congenital hyperpigmentation of the retinal epithelium is seen in Gardner's syndrome. Lester irides are seen in Nail-Patella syndrome.

Answer 310

►A This patient has PAPA (pyogenic arthritis-pyoderma gangrenosum-acne) syndrome. The gene mutated is PSTPIP1, also known as CD2 binding protein 1 (CD2BP1) which encodes prolineserine- threonine phosphatase-interacting protein 1. NOD2 is the gene involved in Blau syndrome; CIAS1 with CINCA/Muckle-Wells/Familial cold autoinflammatory syndromes; AIRE with APECED (autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy); FOXP3 with IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X -linked) syndrome.

Answer 311

►A Granuloma annulare is the entity pictured. Subtypes include localized GA, generalized GA, macular GA, subcutaneous GA, and perforating GA. The subcutaneous variant is most common in children; there is often a history of trauma to the area. Localized lesions never u lcerate and heal without scarring; 75% of localized lesions clear within 2 years. The perforating variant classically involves the dorsa of the hands. Diabetes is present in 20% of patients with the generalized variant.

Answer 312

►D Approximately 70 % of psoriatic arthritis is asymmetric oligoarthritis. The slide depicts arthritis mutilans, which occurs in about 5% of patients with psoriatic arthritis.

Answer 313

►E Ducas and Kapetanakis pigmented purpura presents as eczematous patches with petechiae and hemosiderin staining. Pruritus is common, and the lesions are often more extensive than the other pigmented purpuras. Histologically, spongiosis is noted. The other listed pigmented purpuric eruptions tend to be asymptomatic, although lesions of Schamberg\'s disease can rarely be associated with itch.

Answer 314

►A The Sign of Lesser-Trelat is defined as the rapidly increasing in size or number of seborrheic keratosis. The associated malignancy is gastric and colon cancer. It may improve with treatment of underlying malignancy.

Answer 315

►E HLA-B17 is associated with an earlier onset and more serious disease of psoriasis. HLA-Cw6 is the most definitive associated HLA type of psoriasis, with a relative risk 9-15 times normal. HLA-B17 amd HLA-B13 are also associated with psoriasis but not psoriatic arthiritis

Answer 316

►A Acute hemorrhagic edema of childhood affects children and infants < 2 years of age. It presents with painful, edematous petechiae and ecchymoses on the head and distal extremities. Facial edema may be the initial sign. Triggering factors include infection, drugs, and immunization. It lacks systemic features and resolves in 1-3 weeks without sequelae.

Answer 317

►C Postherpetic neuralgia (PHN) is a common complication seen in herpes zoster. Defined as the presence of pain after skin lesions have healed. Occurs in 10-15% of patients but incidence may increase to 50% in patients over the age of 60 who develop herpes zoster. In most cases PHN resolves within the first 12 months but may persist for years. While generally self -limited, antiretroviral therapy is recommended for patients over the age of 50, those who are immunocompromised, or have ophthalmic zoster.

Answer 318

►D The most definitive treatment for symptomatic ocular rosacea is an oral tetracycline.

Answer 319

►B Cyclosporine is highly effective in most patients with severe chronic plaque-type psoriasis. Doses start at 2.5 to 4mg/kg/day and can go as high as 5.5mg/kg/day. Renal impairment may occur and is often reversible. If the creatinine increases 30% or greater from baseline the dose should be reduced by 25% and the patient followed closely.

Answer 320

►A Anti-Jo-1 (histidyl TRNA syntetase) antibodies are seen in a minority of patients with Dermatomyositis and are associated with pulmonary fibrosis. Anti-Jo-1 Abs are also associated with Raynaud's and polyarthritis. Treatment of dermatomyositis may include systemic corticosteroids, antimalarials, methotrexate, azatioprine and photoprotection.

Answer 321

►E Type III (mixed) cryoglobulinemia consists of rheumatoid factors that are polyclonal IgM and IgG, complexed with each other or with protein. Type II (mixed) cryoglobulinemia consists of monoclonal IgM rheumatoid factor complexed with polyclonal IgG. Type I cryoglobulinemia consists of a single monoclonal immunoglobulin, usually IgG or IgM, usually due to an underlying B-cell malignancy (myeloma or lymphoma).

Answer 322

►E Vitamin B12 can cause acneiform eruptions.

Answer 323

►A This patient has Sweet's syndrome or Febril neutrophilic dermatosis. The most common cancer associated is acute myelogenous leukemia. it is also associated with lymphoma and polycythemia vera. It is also associated with anemia, leukocytosis, neutrophilia, and elevated ESR.

Answer 324

►E Nevus achromicus is another name for nevus depigmentosus. It usually presents at birth or appear during early infancy as normal pigmentation increases. Most individuals will have a solitary lesion of nevus depigmentosus, but multiple lesions and segmental forms of nevus depigmentosus have been described. Nevus depigmentosus tends to persist lifelong, but remains unchanged after onset. The hypopigmented white spots of tuberous sclerosis are most difficult to distinguish from nevus depigmentosus, but lack of other cutaneous or systemic manifestaions exclude tubrous sclerosis. Lesions of vitiligo tend to be depigmented (melanocytopenic not melanopenic as in the question), and show a bright white coloration with Wood's lamp examination. Nevus anemicus is a distinct vascular birthmark characterized by blanching of cutaneous blood vessels, hence presenting as a "white" patch of skin that becomes unnoticeable when the surrounding skin is blanched with a glass slide ("diascopy").

Answer 325

►A Serum ACE levels are positive in only approximately 60% of patients with sarcoidosis. However the specificity is only 80%. Therefore, serum ace levels are less helpful in diagnosis (they can be helpful in following treatment response.) For example, if your pretest clinical suspicion of sarcoidosis is 25% (i.e. 1 of 4 likely possible etiologies based on differential diagnosis), then of a 1000 patients tested, 250 will have sarcoidosis. 160 of these will be detected by an abnormal serum ace level, but 150 of the non-sarcoidosis patients will also have an abnormal ace level giving you a positive predictive value of just over 50% making it a poor diagnositic test.

Answer 326

►C Only testosterone and dihydrotestosterone bind the androgen receptor, thus adrenal androgens (androstenedione and dehydroepiandrosterone) virilize only in so far as they serve as precursors for testosterone and dihydrotestosterone.

Answer 327

►A In patients with rosacea, patients experience rhinophyma that is from the sebaceous hyperplasia. Initially the nose becomes swollen with prominent pores followed by fibrosis in the later stages.

Answer 328

►B RNP antibodies target small ribonucleoproteins. These include SS-A (Ro), SS-B (La), Sm, and U1RNP. The total amount of antibody has more diagnostic value than the mere presence of antibody.

Answer 329

►A Eosinophilic pustular folliculitis is a noninfectious eosinophilic infiltration of hair follicles. The classic case is mainly from Japanese men. It is also immunosuppression association.

Answer 330

►D Occasionally epidermotropic T-cells are seen in the lichenoid reaction and thus may raise concern for mycosis fungoides. Lichen planus pigmentosus inversus, in particular, presents in classic sites of mycosis fungoides, including the axilla, inguinal, and inframammary areas. The individual lesions of lichen planus pigmentosis are typically smaller, however, than those encountered in mycosis fungoides, thus helping with the differential. Lesions of lichen planus pigmentosus are hyperpigmented, with most cases prsenting in skin of color. Oral involvement is rare and the trunk can be involved.

Answer 331

►C While most AD patients do not have food allergy, food allergens exacerbate AD in at least a subset of patients, particularly infants and young children. Eggs, milk, peanuts, soybeans, tree nuts, fish, and wheat are the most common food allergens implicated. Dust mites (Dermatophagoides pteronyssimus) are among the environmental allergens that may exacerbate AD.

Answer 332

►A Takayasu arteritis is a large vessel vasculitis that manifests as progressive granulomatous inflammation of the aorta and its major branches. The systemic vasculitidies are classified into three categories: large vessel, medium-sized vessel and small vessel vasculitis. Takayasu arteritis and Giant cell (temporal) arteritis are the two large vessel vasculitidies. Polyart eritis nodosa and Kawasaki disease are medium-sized vasculitidies. Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, Henoch-Schonlein purpura and cutaneous leukocytoclastic vasculitis are examples of small vessel vasculitidies.

Answer 333

►D Mast cell mediators can be grouped into two classes: preformed and newly formed. Preformed mediators include tryptase, histamine, serotonin, and heparin. Newly formed mediators include prostaglandin D2, leukotriene C4, and platelet activating factor.

Answer 334

►A Penicillamine induces native systemic lupus erythematosus, associated with anti-dsDNA antibodies, in contrast to drug-induced lupus erythematosus which has been associated with exposure to hydralazine, procainamide, sulfonamides, penicillin, anticonvulsants, minocycline, and INH and is associated with anti-histone antibodies.

Answer 335

►A Sneddon- Wilkinson disease, also know as subcorneal pustular dermatosis classically presents with what has been described in this question. SAPHO syndrome is a mnemonic which stands for synovitis, acne conglobata or fulminans, pustular psoriasis, hyperostosis and osteitis. Transient pustular melanosis is seen in neonates. Pthirus pubis is pubic lice.

Answer 336

►A Monselâ€™s solution is also known as ferrous subsulfate and can be a cause of iatrogenic tattooing.

Answer 337

►A Guttate psoriasis refers to a distinctive, acute clinical presentation of an eruption characterized by small, droplike, 1-10 mm in diameter, salmon-pink papules, usually with a fine scale. It is more commonly seen in individuals younger than 30 years with a history of upper respiratory infection that precedes the eruption by 2-3 weeks. The most common organism is group A beta-hemolytic streptococci (eg, Streptococcus pyogenes). Although recurrent episodes may occur, especially those due to pharyngeal carriage of streptococci, isolated bouts are known to occur. Guttae psoriasis may also occur with other infections or in isolation without any identifiable infection.

Answer 338

►C This slide shows blue-gray discoloration at the anterior shins and is characteristic of minocycline hyperpigmentation.

Answer 339

►D Taxane probably cause nail changes more commonly than other drugs. Cutaneous toxicity has been reported with taxanes and includes erythema and desquamation, involving primarily the hands. Taxanes exert their cytotoxic effect by reversibly binding the α-subunit of tubulin, thereby inducing tubulin polymerization and inhibiting microtubule depolymerization. A balance between polymerization and depolymerization is needed for normal microtubule function.Taxanes disrupt this balance, leading to arrest at the G2/M phase of the cell cycle.

Answer 340

►A Drug induced dermatomyositis is the most common cause is hydroxyurea. Other common causes are statins, cyclophosphamide, and terbinafine.

Answer 341

►D Anti-155/140 has been associated with an increased risk of malignancy in dermatomyositis patients. Anti-SRP is associated with fulminant dermatomyositis/polymyositis and cardiac involvement; anti- Mi2 is associated with the shawl sign, periungual telangiectasias, cuticular overgrowth, and Gottrons papules; anti-Jo1 and anti-PL-7 are associated with antisynthetase syndrome and interstitial lung disease.

Answer 342

►E The Dunnigan variant of partial lipodystrophy is characterized by normal appearance at birth followed by a gradual loss of subcutaneous tissue from the arms and legs around the time of puberty. The genetic defect involves the gene encoding lamins A and C (LMNA) which are intermediate filaments integral to the nuclear envelope. The AGPAT2 and seipin genes are mutated in type I and type II congenital generalized lipodystrophies, respectively. The gene for zinc metalloproteinase (ZMPSTE24) is mutated in mandibuloacral dysplasia.

Answer 343

►A Patients that are on beta blockers can be at risk for precipitating gutate psoriasis. Streptococcus can also precipitate gutatte psoriasis.

Answer 344

►A 80% of cases of erythema gyratum repens have been associated with underlying malignancy. Lung cancer is the most common neoplasm. The skin eruption preceedes the detection of malignancy by an average of 9 months

Answer 345

►E Macroglossia is not a feature of Behchets disease. Macroglossia, a classic feature that occurs in about 20% of patients with primary systemic amyloidosis due to deposition of amyloid in the tongue leading to firm and enlarged tongue. These patients can also have hemorrhagic papules, plaques and blisters on its surface. Macroglossia is seen in many diseases and syndromes including Down's syndrome, mucopolysaccharoidoses, cretinism, hypothyroidism, lipoid proteinosis and Beckwith-Wiederman Syndrome.

Answer 346

►A The eruptive type of syringomas. In addition to the description above, some of the cysts have small comma-like tails, which is known as a tadpole pattern. Eruptive syringomas are histologically identical to those on the eyelid, but appear suddenly on the neck, chest, axillae, upper arms, and periumbilically. It usually occurs in young people. The other options do not have an eruptive form and tend to occur in different locations of the body other than the arms and thorax.

Answer 347

►D Hand-Schuller-Christian disease is a chronic multifocal form of Langerhans cell histiocytosis.70% of cases occur between the ages of 2 and 6. The four characteristic clinical findings are bone lesions, diabetes insipidus, exophthalmus, mucocutaneous lesions. Bones lesions are osteolytic and preferentially involve the calvarium.

Answer 348

►B Acquired partial lipodystrophy (Barraquer-Simmons Syndrome) is characterized by an insidious progressive loss of fat that usually begins in the face and scalp and progressed downward. Most patients with this form of lipodystrophy have reduced levels of C3 resulting from circulation polyclonal IgG called "C3 nephritic factor" which results in uncontrolled activation of C3 and contributes to renal damage.

Answer 349

►A Churg-Strauss syndrome has six diagnostic criteria as set by the American College of Rheumatology: Blood Eosinophilia, Asthma, Neuropathy, Sinus abnormalities, Allergies and Perivascular eosinophils. The presence of four of these six criteria yields a diagnostic sensitivity of 85% and specificity of 99.7%.

Answer 350

►E Cigarette smoking accelerated photoaging. Solar elastosis in patients with significant smoking history is present not only in the papillary dermis, but also the reticular dermis. It has also been shown to increase the incidence of skin cancer, decrease the survival of surgical grafts, increase the severity of wrinkling.

Answer 351

►C Common sites for chloracne include the malar cheek, the post-auricular scalp and in men, the scrotum.

Answer 352

►B Psoriasis has HLA associations with all of these HLA types. HLA-B17 is associated with earlier onset and more serious disease.

Answer 353

►B Medications that have been reported to cause flares of psoriasis include antimalarials, beta blockers, NSAIDS, penicillin, tetracycline, ACE inhibitors, G-CSF, interferons and lithium. In addition, withdrawal from systemic corticosteroids can induce a flare.

Answer 354

►A Extracutaneous manifestations of scleroderma include esophageal dysmotility, pulmonary fibrosis with resultant pulmonary hypertension, cardiac involvement with conduction defects, pericarditis or heart failure, and renal involvement with acute renal failure. Esophageal dysmotility is the most common systemic manifestation with up to 90% of systemic sclerosis patients affected. Dysphagia can precede cutaneous involvement thereby giving rise to the term systemic sclerosis sine scleroderma. Dysmotility is manifested by reduced peristalsis, especially in the lower two-thirds of the esophagus. This can be demonstrated on a radiologic or manometric study.

Answer 355

►A Carotenoderma is yellowish discoloration of the skin secondary to carotenemia. Carotene is excreted by sebaceous glands and in sweat, so the yellow pigmentation appears first on the face (especially nasolabial folds and forehead) and then becomes diffusely distributed with accentuation in palms and soles. In contrast to jaundice, carotenoderma spares mucous membranes and sclera

Answer 356

►E The image shows symmetrical erthematous to violaceous plaque on dorsal hands with some periungual erythema and telangiectasia. Skin biopsy was consistent with interface dermatitis and mucin. Many drugs have been reported to cause dermatomyositis-like picture, which include hydroxyurea, D-penicillamine, statins, phenytoin and alfuzosin (alpha antagonist for BPH). Captopril is not associated with DM-like rash.

Answer 357

►A Anti-Mi-2 antibodies correlate with shawl sign, cuticular changes (as seen in the image) and an overall favorable prognosis. Anti-Jo-1 antibodies correlate with pulmonary fibrosis, Raynaud's and polyarthritis. Anti-SRB antibodies correlate with cardiac disease and a poor prognosis. AntiKU antibodies correlate with sclerodermatomyositis. Anti-PL7 and Anti-PL12 antibodies (antisynthetase antibodies) also correlate with pulmonary disease.

Answer 358

►D Drug-induced lupus erythematous has been associated with exposure to hydralazine, procainamide, sulfonamides, penicillin, anticonvulsants, minocycline, and INH. It generally has a benign course. The presence of anti-histone antibodies are closely associated with symptomatic disease. Hydralazine and procainamide are common culprits, with slow acetylators of hydralazine (HLA- DR4) more prone.

Answer 359

►A The most common form of cutaneous mastocytosis is urticaria pigmentosa. It is diagnosed by measuring serum tryptase levels. The new diagnostic method is measurement of tryptase in bonemarrow which is a new sensitive marker of the mast cell.

Answer 360

►A Type I cryoglobulinemia is associated with monoclonal IgM. Other associated malignancy are multiple myeloma and Waldenstroms macroglobulinemia. The lab findings are elevated cryoglobulins and on pathology there are eosinophilic intravascular deposits.

Answer 361

►C P. acnes flourishes in the presence of sebum and makes an enzymatic lipase which cleaves triglycerides into free fatty acids. Free fatty acids serve as an indirect measure of P. acnes populations on the skin. P. acnes itself is also pro-inflammatory; it can activate complement, as well as neutrophil chemotaxis and activity. Il-1, a pro-inflammatory cytokine, may promote follicular plugging and microcomedo formation. TLR-2 activation by P. acnes leads to stimulation of pro- inflammatory cytokines.

Answer 362

►A Arteriography is the technique of choice to evaluate arteriovenous malformations. Doppler should be done at age 1 and annual xray after the age of 2. Multi-detector CT and fast 3D MRI venography can also be done.

Answer 363

►E Ong et al found a deficiency of HBD-2 and LL 37, which are innate antimicrobial peptides, in lesions from patients with atopic dermatitis compared to lesions from patients with psoriasis. IL4 and IL-13, which are Th2-type cytokines, were found to inhibit expression of HBD-2 and LL 37. Decreased expression of HBD-2 and LL 37 may account for the increased susceptibility to S. aureus infection in patients with atopic dermatitis.

Answer 364

►C AL protein is associated with primary systemic amyloid. AA is associated with secondary systemic amyloid. AB2M is associated with hemodialysis. AB is associated with alzheimer‘s disease. Altered keratin is associated with lichen and macular amyloid.

Answer 365

►E Erythromycin inhibits the hepatic cytochrome P450 system and can increase serum levels and potential toxicities of carbamazepine, theophylline, warfarin, digoxin, and methylprednisolone.

Answer 366

►D Langerhan cell histiocytosis includes the diseases Letterer-Siwe, Hand-Schuller-Christian, Eosinophilic Granuloma, and Hashimoto-Pritzker. Cells stain positively with CD1a, S100, langerin, and peanut agglutinin. Cells may also be indentified by the presence of Birbeck granules.

Answer 367

►A Morbihan's Disease, also known as rosacea lymphedema or persistent edema of rosacea, presents with hard, nonpitting edema. It is often misdiagnosed as cellulitis.

Answer 368

►D Several drugs have been incriminated as inducers of psoriasis, in particular lithium, B-Blockers, antimalarials, and interferon. More recent additions include terbinafine, calcium channel blockers (nicardipine, nifedipine, nisoldipine, verapamil, and diltiazem), captopril, glyburide, and lipi- lowering drugs such as gemfibrozil.

Answer 369

►D Tacrolimus and pimecrolimus are non-steroid anti-inflammatory drugs and are not associated with side effects reported with use of topical corticosteroids, including atrophy, striae formation, telangiectasias, cataracts, and HPA axis suppression.

Answer 370

►A The patient has steatocystoma multiplex that is linked to the defect in keratin 17, 10. The lesions usually occur in the second and third decades of life. It also occurs sporadically and is inherited in the benign form.

Answer 371

►D This patient has mild-moderate inflammatory acne. Azelaic acid is the only medication listed which falls under category B. The others listed are category C, except for tazarotene, which is category X

Answer 372

►C Xanthelasma are the most common type of xanthoma. They are usually present without any other disease, but may occur concomitantly with other xanthomas, and can occur in types II and III hyperlipoproteinemias (familial hypercholesterolemia, common hypercholesterolemia, and familial dysbetalipoproteinemia). They are also common among women with biliary or hepatic disoders, and are also seen in myxedema, diabetes, and phytosterolemia.

Answer 373

►A IgG monoclonal gammopathy is seen in over 80% of cases of necrobiotic xanthogranuloma. Pathology reveals palisading granulomas with necrobiosis and cholesterol clefts. Recurrence rates as high as 42% have been reported after surgical excision.

Answer 374

►A While acute atopic dermatitis is a TH2 state, chronic eczematous AD is most often a TH1 cytokine environment with interferon-gamma being the best choice. Initial lesions TH2 dominant, cytokines IL-4,5,10,13. IL-4/5 produce elevated IgE and eosinophilia, IL-10 inhibits delayed-type hypersensitivity. IL-4 downregulates IFN-gamma.

Answer 375

►B Sarcoid is granulomatous autoimmune condition characterized by the formation of non-caseating granulomas which may affect any organ system. Lacrimal gland involvement occurs in 15 -28% of patients. Manifestation of lacrimal gland involvement includes bilateral painless swelling.

Answer 376

►C The patient has Type 2 minocycline-associated hyperpigmentation. Three types of minocyclineassociated hyperpigmentation are generally described. The first is blue-black discoloration appearing in areas of prior skin injury, such as acne scars. The second type is a blue- gray discoloration, often on the lower anterior legs and forearms. The third type is the least common, and is characterized by muddy brown discoloration of sun-exposed areas. The first two types show staining for both iron and melanin (Fontana Masson stains melanin black; Perls stains iron [hemosiderin] blue). The third type shows increased melanin at the basal layer and within macrophages.

Answer 377

►A The National Psoriasis Foundation Consensus states that patients with a positive TB. Treat latent TB prior to starting therapy. After 1-2 months of appropriate TB treatment, psoriasis therapy may begin is necessary.

Answer 378

►E This slide shows a woman with rosacea. Green and red are on opposite sides of the color wheel and thus can ―cancelǁ each other out. Green concealer can neutralize redness on the skin.

Answer 379

►C Menopausal flushing occurs at menopause or perimenstrual when estrogen levels are low. Pharmacologic menopause may be caused by drugs, including danazol, tamoxifen, clomiphene citrate, decapeptyl, leuprolide, and 4-hydroxyandrostenedione. Treatment is with oral estrogen replacement or clonidine hydrochloride 0.05mg bid. Nadolol is an effective treatment of emotional flushing.

Answer 380

►A Mycosis fungoides is the most common form of cutaneous T cell lymphoma. It can look like chronic eczema and can be histologically very difficult to distinguish from mycosis fungoides. CA27.29 may be a marker for advanced mycosis fungoides.

Answer 381

►A This patient has fibrodysplasia ossificans progressiva characterized by endochondral bone formation (the other types have intramembranous bone formation), noggin gene defects and other systemic symptoms as listed. This is a progressive and potentially fatal condition. Progressive osseous heteroplasia is also progressive, seen in mostly females, and demonstrates increased alkaline phosphatase, LDH and CPK with normal calcium, phosphate, and PTH. Platelike osteoma cutis is limited and seen in children and newborns. Albright's hereditary osteodystrophy is due to a mutation in GNAS-1. This disorder is characterized by a lack of responsiveness to parathyroid hormone, resulting in low serum calcium, high serum phosphate, and appropriately high serum parathyroid hormone. Individuals with Albright's hereditary osteodystrophy have short stature, characteristically shortened fourth and fifth metacarpals, rounded facies, and often mild mental retardation. Calcinosis cutis is not a form of osteoma cutis.

Answer 382

►D Other bactericidal drugs include bacitracin, monobactams, quinolones, vancomycin, and polymyxin B. Clindamycin, tetracycline, trimethoprim, and chloramphenicol and bacteriostatic.

Answer 383

►B The question stem describes a case of granuloma faciale. This condition presents with red-brown papules and plaques on the face, most commonly in middle-aged Caucasian males. While traditional treatment options include intralesional steroids with or without cryotherapy, pulse dye laser (595 nm) represents another treatment option for this condition, with successful clearance in a number of case reports.

Answer 384

►E Well-documented adverse effects and toxicities of cyclosporine include renal impairment , hypertension, elevated triglycerides, hyperkalemia, hypomagnesemia, hepatotoxicity, hypertrichosis, and long-term increased risk of malignancy.

Answer 385

►C The presence of EN, bilateral hilar lymphadenopathy, uveitis, fever, and arthritis in patients with sarcoidosis is known as Lofgren's syndrome. Heerfordt's syndrome, also known as uveoparotid fever, frequently occurs in patients with sarcoidosis of the central nervous syndrome. It consists of uveitis, facial nerve palsy, fever, and parotid gland involvement. Milulicz's syndrome is bilateral sarcoidosis of the parotid, submandibular, sublingual, and lacrimal glands. Loeffler's syndrome is characterized by a patchy infiltrate in the lungs and eosinophilia of the blood and sputum. Loeffler's syndrome can be with creeping eruption (larva migrans). Schnitzler's syndrome is a rare disorder of chronic urticaria, fever, disabling bone pain, hyperostosis, increased erythrocyte sedimentation rate, and monoclonal IgM gammopathy. Pruritus is not a feature of Schnitzler syndrome.

Answer 386

►A Absolute ethanol or Carnoy's fluid allows visualization of urate crystals. The crystals are brown and refractile with polarized light.

Answer 387

►B Hypertrophic lichen planus is a type of lichen planus characterized by hyperkeratosis. They are usually pruritic and often found on the extremities. Civatte bodies are degenerated keratinocytes often seen in lichen planus.

Answer 388

►B The correct answer is surgery and itraconazole, which inhibits 14-alpha-demethylase. Terbinafine inhibits squalene epoxidase. Griseofulvin disrupts microtubule mitotic spindle formation. Caspofungin inhibits synthesis of beta-1,3-diglucan. Foscarnet inhibits fungal cytochrome P-450 mediated 14 alpha-lanosterol demethylation.

Answer 389

►B This patient has pustular psoriasis of pregnancy which is also called "impetigo herpetiformis." It is characterized by red plaques with a peripheral ring of pustules that are distributed symmetrically in flexural areas and trunk and extremities. Patients can have elevated ESR and leukocytosis as well as hypocalcemia. There is a risk of fetal morbidity and mortality secondary to placental insufficiency and maternal mortality secondary to cardiac or renal failure. Premature labor may be associated with cholestasis or pregnancy. Patients with herpes gestationis may have a risk of premature labor and small for gestational age. Microcephaly may occur with isotretinoin taken during pregnancy.

Answer 390

►A Dialysis related amyloidosis is due to beta 2-microglobulin. The amyloid fibril is altered by uremia and causes carpal tunnel, bone cyst, and spondyloarthropathy.

Answer 391

►C This patient has a presentation consistent with industrial acne. Dioxin (2,3,7,8 tetrachlorobenzodioxin) is a well-known, potent trigger of this acneiform eruption. Overall, insoluble cutting oils are the most frequent cause of industrial acne.

Answer 392

►A Triglyceride is the major constituent of sebaceous lipid, accounting for over 50% of the lipid. Wax esters constitute about 25% of sebaceous lipid and Squalene accounts for about 15%. The remainder is free cholesterol and cholesterol esters.

Answer 393

►A Patients that have vascular malformations can experience localized intravascular coagulation. Less then 85% will experience this with segmental pattern. The risk factors are large surface areas, muscle involvement, palpable pheboliths. The treatment is low molecular weight heparin.

Answer 394

►E Both the congenital (Dunnigan - Type 1) and acquired (Barraquer-Simons) lipodystrophies are caused by mutations involving the nuclear lamina of the cell. Dunnigan lipodystrophy is characterized by a mutation in LMNA and Barraquer-Simons is caused by a LMNB2 mutation.

Answer 395

►E Psoriatic nail changes may be of nail matrix or bed in origen. Pits are the most common findings, while splinter hemorrhages are the least. Psoriatic nail changes involving the matrix include pits (representing focal psoriasis of the proximal nail matrix) and leukonychia.

Answer 396

►E Acute hemorrhagic edema of childhood affects children and infants < 2 years of age. It presents with painful, edematous petechiae and ecchymoses on the head and distal extremities. Facial edema may be the initial sign. Triggering factors include infection, drugs, and immunization. It lacks the systemic features of HSP, and resolves in 1-3 weeks without sequelae. HSP occurs mostly in children. There is an antecedent URI in 75% of cases. HSP involves the skin, synovia, GI tract, and kidneys. Long-term morbidity results from renal disease, which is predicted by the spread of purpura to the upper trunk.

Answer 397

►D Barraquer-Simons syndrome, acquired partial lipodystrophy, presents typically in females and is secondary to a mutation in LMNB2. It is often preceded by a viral illness. More than one third of patients will have glomerulonephritis, which may lead to chronic renal sequelae.

Answer 398

►C Recommended dietary allowance of vitamin D as established by the Institute of Medicine in 2010 is 400 international units for infants 0 to 12 months, 600 IU for ages 1 to 70 years, and 800 IU for ages 71 and above. Additionally, recommended allowance for pregnant/lactating women is 600 IU.

Answer 399

►A Acute intermittent porphyria has a defect in porphobilinogen deaminase. Acute intermittent porphyria (AIP) is a rare autosomal dominant metabolic disorder affecting the production of heme, the oxygen-binding prosthetic group of hemoglobin.

Answer 400

►A Lichen planus classically involves the wrists, ankles, and oral mucosa. It is characterized by violaceous polygonal, purple, pruritic papules. It has a strong association with hepatitis C virus. Many other associations exist, including other viruses and medications, but hepatitis C is the best choice.

Answer 401

►D The history and clinical appearance of this patient suggest a possible hormonal basis to her acne. The work-up in answer d assesses for adrenal hormonal production and screens for PCOS. Androstenedione is of ovarian origin.

Answer 402

►A Acne fulminans is an explosive form of acne in which patients may develop systemic symptoms, ulcerated nodules on the face and trunk. Acne fulminans may be triggered by initiation of isotretinoin therapy. The treatment of choice is systemic steroids at a dosage of 1 mg/kg/day tapered over the course of 6 weeks.

Answer 403

►B This patient has pellagra secondary to isoniazid treatment. He has the photosensitive eruption and Casal's necklace *, in addition to diarrhea and depression. Other symptoms include the 3 D 's: dermatitis, diarrhea, dementia. Other potential medications that may cause this constellations of symptoms include azathioprine and 5-FU.

Answer 404

►A Pyoderma gangrenosum, (PG), is an uncommon, ulcerative inflammatory skin condition characterized by boggy ulcerations with undermined borders preceded by the breakdown of a painful nodule or pustule. These lesions characteristically enlarge progressively over time and display marked tenderness. The diagnosis of PG is a diagnosis of exclusion and infectious etiologies should be excluded. Pyoderma gangrenosum can arise in the absence of an underlying disorder or it can be seen in association with underlying systemic conditions. These conditions include inflammatory bowel disease such as ulcerative colitis and Crohn's disease, polyarthritis, chronic active hepatitis and Behcet's disease. Pyoderma gangrenosum has also been associated with an underlying paraproteinemia, mostly of the IgA type, although IgM and IgG types have also been described. In addition, some patients have myeloma at presentation or develop it subsequent to their diagnosis of PG. Pyoderma gangrenosum has also been described in association with myelodysplasia, and in acute myeloblastic, myelomonocytic, and chronic myeloid leukemia. Treatment of pyoderma gangrenosum should include therapy directed at the underlying systemic disorder.

Answer 405

►A The most common allergen in cosmetic products is quaternium 15. It is a preservative in personal care products and cosmetics.

Answer 406

►A Treatment of Raynaud's includes calcium channel blockers such as nifedipine 30-6-mg/d, antiplatelet aggregation drugs (such as aspirin or dipyridamole), pentoxyphilline 400mg BID TID and D-penicillamine. Losartan 50mg/d may reduce frequency and severity of Raynaud's.

Answer 407

►A Patients with Muckle-Wells has amyloid AA fibrils. It is an autosomal dominant condition characterized by deafness, hives, and mutation in the CIAS1 gene.

Answer 408

►B 20% of patients that are diagnosed with multicentric reticulohistiocytosis have a risk of developing a malignancy but there is no predominant type. Pathology shows nodular infiltrate composed of multinucleated oncocytic giant cells with eosinophilic cytoplasm.

Answer 409

►A A patient with this condition has a defect in keratin 17. These conditions can be seen together since they share the same defect in the genotype. Both pachyonychia congenital type 2 and steatocystoma multiplex have a defect in keratin 17.

Answer 410

►A Patients with amyloidosis has an association malignancy with multiple myeloma and plasma cell dyscrasia. The pathology is eosinophilic, amorphic fissured globules in the dermis. The amyloid is composed of immunoglobulin lambda light chains.

Answer 411

►D Primary sytemic amyloidosis involves the skin in 40% of cases. The tongue, heart, and GI tract are commonly involved. The protein AL is derived from Ig light chains (lambda subtype); AL is also found in nodular or tumefactive cutaneous amyloidosis produced by a plasmacytoma. Glossitis is common, and may lead to dysphagia; the lateral aspects of the tongue often show indentations from teeth. Purpuric lesions result from amyloid infiltration of blood vessels, and occur after trauma (pinch purpura). Bullous amyloidosis presents with tense, hemorrhagic bullae at areas of trauma; lesions are subepidermal. Arthropathies of small joints, enlarged deltoids (shoulder pad sign), factor IX and X deficiencies, cardiac arrythmias, and CHF may all result.

Answer 412

►A Human sebum is rich in triglycerides. The lipase made by P. acnes cleaves triglycerides to free fatty acids.

Answer 413

►B Linea nigra is a hyperpigmented, linear patch that often becomes apparent during pregnancy. It is thought to be secondary to hormonal fluctuations and usually spontaneously resolves after pregnancy. Voight and Fuchter lines delineate a vestigial hyperpigmentation of the dorsal aspect of organisms.

Answer 414

►B In dermatitis herpetiformis, antibodies are found to transglutaminase 3, and the direct immunofluorescent studies show granular IgA and C3 in the dermal papillae. Antibodies to BPag2 are found in bullous pemphigoid. Mutations in plectin are found in EBS with muscular dystrophy. Mutations in laminin 5 are found in patients with JEB,Herlitz type. Mutations in transglutaminase I are found in pateints with lamellar ichthyosis and non bullous congenital ichthyosiform erythroderma.

Answer 415

►D Phrynoderma or ―toad skinǁ* is typically associated with vitamin A deficiency. These keratotic follicular papules often first develop on anterolateral thighs and posterolateral upper arms then spread to extremities, shoulders, abdomen and back. Although phrynoderma is originally reported in association with vitamin A deficiency, it can also be observed with defeciencies in Bcomplex vitamins and vitamins C and E, in addition to essential fatty acid deficiency.

Answer 416

►A The first step in the formation of a microcomedone is the obstruction of the pilosebaceous unit by keratinocytes. After that has occured, increased sebum and P. acnes can contribute to the further formation of the comedone.

Answer 417

►A Patients with Sturge-Weber have seizures as a common neurologic abnormality. They can have glaucoma in 2/3 of patients at birth. Tram track calcifications don't appear on XR before two years old.

Answer 418

►A Lofgren syndrome is characterized by fever, erythema nodosum, bilateral hilar adenopathy, and arthralgias. It is associated with anterior uveitis in 6% of patients.

Answer 419

►B The patient has Reiter's syndrome. Reiter's syndrome is a chronic inflammatory disease similar to psoriasis with psoriatic arthritis, and is thought to be a variant form. The classic triad consists of urethritis, conjunctivitis, and arthritis. Few patients present with the classic triad, and thus the syndrome can be diagnosed with peripheral arthritis >1 month duration and associated urethritis (or cervicitis). It occurs in young men with the HLA-B27 genotype and rarely occurs in women. Skin findings include keratoderma blennorrhagicum and circinate balanitis (in men), as well as oral erosions, severe stomatitis, and nail changes. The course of disease is marked by exacerbation and remission; a chronic deforming arthritis occurs in 20%. Treatment includes topical steroids, NSAIDs, methotrexate, acitretin, cyclosporine, and TNF -inhibiting biologics, such as etanercept.

Answer 420

►D It is a rare entity. Patients complaint of edematous and erythematous plaques, usually on the trunk. The lesions generally respond to antimalarials.

Answer 421

►D Sneddon-Wilkinson disease, or subcorneal pustular dermatosis, presents with superficial pustules in annular and serpiginous patterns in the axillae, groin, and abdomen. Middle-aged women are most often affected. This condition rarely occurs in association with an IgA monoclonal gammopathy. It is a chronic condition, possibly related to psoriasis, with remissions of variable duration. Treatments including dapsone, acitretin, and narrow band UVB.

Answer 422

►E Dermatomyositis is an autoimmune condition which presents with typical skin findings and muscle weakness. Age-appropriate screening should be done for internal malignancy as there is a higher incidence of cancer in these patients. Polymorphisms of tumor necrosis factor-alpha have been implicated in the etiology. Interferon alpha has also been implicated.

Answer 423

►E Gottron's papules and periungual telangiectasias (Samitz's sign) are among the cutaneous features of dermatomyositis, which also include heliotrope rash, photosensitive poikiloderma of the upper back (shawl sign). Anti-Jo-1 antibody is present in 20-30% of these patients. The target antigen is anti- histidyl-tRNA synthetase (Jo-1). The presence of this antibody corresponds to the development of pulmonary disease.

Answer 424

►A HLA-Cw6 has a relative risk of 9-15 times the normal risk for psoriasis. HLA B-17 is associated with early onset and more serious disease.

Answer 425

►C Relapsing polychondritis is a rare disease manifested by recurring inflammation of cartilagi nous tissue. Antibodies to type II collagen are thought to be pathogenic in this disease. Clinically, patients have auricular chondritis and arthritis. The chondritis is limited to the cartilaginous portion of the external ears. Involvement of the cartilaginous portions of the eye, respiratory tract, the inner ear, and the cardiovascular system has been reported as well. A significant portion of patients with relapsing polychondritis have an associated rheumatic or autoimmune disease.

Answer 426

►E Dioxin, halogen bromide, iodide, androgenic hormones such as testosterone can cause acneiform eruption. Tazarotene is a topical retinoid used to treat acne.

Answer 427

►A The celery is a common photosensitizing agent. It can cause both a phototoxic and photoallergic reactions. Celery and parsnip and weeds also can cause these reactions.

Answer 428

►E Pseudotumor cerebri, or benign intracranial hypertension is more common in adolescent and young adult women, but can occur in children. Medicines reported to be associated with the condition include vitamin A analogues, tetracyclines, steroids (especially in withdrawal), nalidixic acid, sulphonamides, lithium, thyroxine, growth hormone, amiodarone and tamoxifen. It most often presents with headache (90% of cases), pulsatile in quality. Less frequent symptoms are visual disturbances and pulsatile tinnitus. Pseudotumor can also be completely asymptomatic. The mechanism is not fully understood but current opinion favors impaired reabsorption of cerebrospinal fluid. Of the medicines associated with the condition, minocycline is most frequently reported in the literature followed by tetracycline and doxycycline. Isotretinion has been repored to cause it and it is possible that the incidence of pseudotumor may increase if two or more drugs which might cause it are used together. For this reason tetracyclines should not be prescribed concomitantly with oral retinoids.

Answer 429

►A Contact lens and ophthalmic solutions have the allergen thimerosal.

Answer 430

►A Patients that have dermatomyositis and scleroderma have roughness, hemorrhages, and necrosis of the cuticles. Patients that have scleroderma have reduced capillary density and avascular areas which alternate with dilated capillary loupes. Systemic lupus erythematosus reveals a normal density of capillaries which are tortuous and dilated.

Answer 431

►C The most common cause of morbidity and mortality in patients with limited systemic sclerosis is pulmonary hypertension. Pulmonary fibrosis is more often associated with diffuse systemic

Answer 432

►B The patient described has TEN. A diagnosis of TEN can be made if the patient presents with epidermal detachment of >30% of the body surface with widespread purpuric macules or flat atypical targets, or if there are large sheets of epidermal detachment involving >10% of the body surface without purpuric macules or target lesions. The SCORTEN scoring system was developed to assess severity of illness and predict mortality in TEN, and is meant to be calculated within the first 24 hours after admission and again on day three. The score is the sum of the following clinical variables: (1) age over 40 years; (2) heart rate >120 beats per minute; (3) the presence of cancer or hematologic malignancy; (4) epidermal detachment involving body surface area >10% on day one; (5) blood urea nitrogen >28 mg/dL (10 mmol/L); (6) glucose >252 mg/dL (14 mmol/L); and (7) bicarbonate >20 mEq/L. The mortality increases sharply with each additional point, with a score of 5 or greater having a 90% mortality.

Answer 433

►C Attached image shows mastocytosis. The disease presents classically with tan-like or red brown macules or papules. There are different forms of cutaneous mastocytosis, most common form is urticaria pigmentosa which can appear in both children and adults and diffuse cutaneous mastocytosis which occur almost exclusively in infants, solitary mastocytoma and telangiectasia macularis eruptiva perstans. Systemic involvement occurs most commonly in adults and bone marrow,lymph nodes, liver, spleen and GIT are among the most common involved systems whereas genitourinary and neuroendocrine systems are least likely to be involved.

Answer 434

►A Up to 10 % of patients with sarcoidosis may have hypercalcemia. Resultant hypercalciuria and nephrolithiasis may lead to renal failure. The other listed lab abnormalities are not typically associated with sarcoidosis.

Answer 435

►D Both Sweet‘s syndrome and atypical pyoderma gangrenosum fall within the category of neutrophilic dermatoses with bullous and ulcerated lesions. Both favor the head and neck and extremities. Both can recur, and both demonstrate a heavy neutrophilic infiltrate on histopathology. Both are associated with infections and hematologic disorders, though atypical PG is more likely associated with hematologic malignancy. The presence of constitutional symptoms such as fever, arthralgia, and arthritis are strongly associated with Sweet‘s and can assist in differentiation between these entities.

Answer 436

►B About 1/3 of case of elastosis perforans serpiginosa occur in patients with other concomitant disorders. All of the above can occur in patients with EPS; however the most common is Down syndrome. One mnemonic to remember the associated disorders is: PROMEDA. This stands for PXE,Rothmund-Thomson, Osteogenesis imperfecta,Marfan syndrome, Ehlers-Danlos syndrome, Down syndrome, acrogeria.

Answer 437

►C The Wong type of dermatomyositis is characterized by erythematous, hyperkeratotic, follicular papules . It can have a limited or generalized distribution. It can evolve into full -blown dermatomyositis with classic cutaneous features. This rare variant bears a striking clinical resemblance to PRP. It can be distinguished on skin biopsy. It has been reported in both adults and children. Some reports suggest the follicular papules are confined to the skin overlying the extensor knees and elbows and this variant may be more common in Asian patients.

Answer 438

►A This patient most likely has elephatiasis nostra, which is a complication where there is swelling and verrucous changes to the skin. The other choices do not describe the changes that you would see. Psoriasis are silvery scales and PPK are thickened heels. Acanthosis nigracans are velvety changes that occur on the axillary region and the neck.

Answer 439

►A Macrolide antibiotics inhibit RNA-dependent protein synthesis by binding to the 50s ribosomal subunit, and include erythromycin and azithromycin. Tetracyclines inhibit RNA-dependent protein synthesis by binding to the bacterial 30s ribosomal subunit. TMP-SMX inhibits bacterial folic acid synthesis. Benzoyl peroxide is a bactericidal agent with direct oxidizing effects. Azeleic acid is a dicarboxylic acid that inhibits tyrosinase; the mechanism of action against P. acnes is not completely understood.

Answer 440

►A The most common allergen found in wrinkle free clothing is formaldehyde.

Answer 441

►C In the complement deficiency syndrome, low C2 and C4 are most commonly seen. Photosensitivity, annular SCLE lesions, and Ro antibody formation are commonly observed.

Answer 442

►C An abnormal paraproteinemia is found in 90% of cases of scleromyxedema, usually IgG lamda.

Answer 443

►A SJS and TEN is a hypersensitivity syndrome characterized by epidermal necrosis. The most common cause is allopurinol.

Answer 444

►A Patients that are allergic to permanent hair dyes are allergic to PPD. The contact dermatitis can present as a erythematous rash on the hands or on the frontal scalp.

Answer 445

►B Nodular amyloidosis presents as single or multiple nodules, often on the extremities. The primary protein component is of the AL type.

Answer 446

►A Neutrophilic dermatoses en plaque are well defined, sharply demarcated intensely red plaques. Myeloma and B cell lymphomas are rarely associated. These resolve with treatment of the gammopathy.

Answer 447

►B Adiposis dolorosa (Dercum\'s disease) is a disorder that usually occurs in obese women 40 to 60 years of age, and it is characterized by pain in adipose tissue that appears to be out of proportion to the physical findings. The pain can be localized to multiple, painful lipomas on lower extremities and knees. This condition is accompanied by swelling of different areas of the body, such as hand and feet, which may be transient. Patients typically have subjective fatigue or confusion, and may have history of depression or emotional instability. The cause of adiposis dolorosa is not known. The cause of the pain is speculated to be due to the pressure on nerves by the adipose tissue deposits. Therapeutic treatments are not very effective, and can involved procedures such as liposuction, systemic corticosteroids, pregabalin, lidocaine, and psychiatric care.

Answer 448

►C Darierâ€™s sign is seen in patients with urticarial pigmentosa, a common form of mastocytosis. Erythema and wheals are commonly elicited with stroking or rubbing secondary to mast cell degranulation. Symptoms can range from very mild (flushing, hives, no treatment needed) to life- threatening (vascular collapse). Asboe Hansen, commonly seen in pemphigus vulgaris, refers to extension of a bulla to adjacent unblistered skin when pressure is put on top of a bulla.

Answer 449

►A The most common cause of allergic contact dermatitis is nickel and jewelry is also another common allergen. Dimethylglyoxime test is used to detect nickel. If you rub on the item the solution turns color from pink to reddish that can indicate a positive reaction.

Answer 450

►B Pyostomatitis vegetans is a pustular, vegetative variant of pyoderma gangrenosum, found in the oral mucous membranes. It is most frequently associated with inflammatory bowel disease.

Answer 451

►E Type VII collagen is found in the sublamina densa and patients with bullous lupus erythematosus have been found to have antibodies to this protein. Bullous LE and EBA share antibasement membrane zone antibodies of identical specificity (type VII collagen) as well as clinical and histologic overlap. A differentiating feature between bullous LE and EBA is that bullous LE has a dramatic response to dapsone.

Answer 452

►A Patients with kaposi sarcoma associated with AIDs can have kaposi sarcoma associated with HHV8. It can present as a erythematous purple plaque.

Answer 453

►C Eyebrows, eyelashes, and vellus hair are not androgen-dependent, thus there is no difference between these areas of hair growth in men and women.

Answer 454

►D Dermatomyositis is an idiopathic inflammatory disease with myositis and characteristic cutaneous manifestations. There is an increased incidence of malignancy in these patients which may precede, occur with or follow the diagnosis of dermatomyositis. The most common form of malignancy in adult women is ovarian cancer. Other malignancies that have been associated include testicular cancer, gastrointestinal, lung and nasopharyngeal carcinomas.

Answer 455

►A Overall, superficial spreading melanomas are most frequently seen. However, acral lentiginous melanoma is most frequently seen in patients with darker skin types. It is seen on the palms, soles, and nail units.

Answer 456

►C Anetoderma is a benign condition caused by focal loss of elastic tissue. Primary or idiopathic anetoderma originates from previously healthy skin with unknown pathogenesis. Various ocular, bony, cardiac, and other abnormalities have been reported with primary anetoderma. Secondary anetoderma occurs after the resolution of an inflammatory disease of the skin.

Answer 457

►A Von Zumbusch type is also known as generalized pustular psoriasis. The sterile pustules is on trunk, extremities including the nail beds, palms, and soles. Pustules arise in highly erythematous skin.

Answer 458

►A Toll-like receptors are a large group of receptors that recognize a variety of bacterial motifs. P. acnes has been shown to activate TLR-2, leading to signal transduction and production of proinflammatory cytokines.

Answer 459

►D Necrobiotic xanthogranuloma is associated with a monoclonal IgG kappa paraproteinemia in approximately 80% of cases. It is less commonly associated with an IgA gammopathy. Myeloma or myelodysplastic syndromes may resultantly occur.

Answer 460

►A Psoriatic arthritis affects 20-40% of patients with psoriasis. Most (~80%) of psoriatic arthritis patients are rheumatoid factor negative. All of the answer choices represent possible presentations of psoriatic arthritis, but asymmetric oligo- or polyarthritis is the most common presentation.

Answer 461

►C Scleroderma is a systemic disease which may affect almost any organ in the body. Cutaneous findings are characterized by symmetric, thickening of the skin. Dyspigmentation may occur with a "salt and pepper" appearance due to perifollicular retention of pigmentation with depigmentation of the interfollicular skin.

Answer 462

►A A speckled ANA pattern correlates with staining of ribonucleoproteins and is seen in Mixed Connective Tissue Disease (MCTD), Systemic Lupus Erythematosus (SLE), systemic scle rosis and Sjogren's syndrome. A nucleolar staining pattern correlates with staining of nucleolar RNA. Centromere staining correlates with staining of kinetochore. A peripheral staining pattern correlates with staining of native DNA and a homogeneous staining pattern correlates with staining of native DNA and histones.

Answer 463

►C The patient pictured has lichen sclerosus. This likely represents an autoimmune phenomenon, as 20% of both men and women have at least one autoimmune disease (vitiligo, alopecia areata, or thyroid disease), and a larger proportion have circulating antibodies. Extragenital lesions most frequently involve the torso and are usually asymptomatic. Childhood onset occurs in 10-15% of cases, and girls outnumber boys 10:1. Genital disease represents 90% of childhood lichen sclerosus.

Answer 464

►A Cadmium sulfide is responsible for the yellow color in tattoos.

Answer 465

►C Androstenedione is produced by the adrenals and the ovaries. Testosterone is produced by the adrenals, ovaries, and by extraglandular conversion of androstenedione and dehydroepiandrosterone.

Answer 466

►D The patient's biopsy demonstrates the corkscrew hairs of scurvy, or vitamin C deficiency, which is characterized by the 4 "H"s: hemorrhagic signs (tender nodules on the lower extremities), hyperkeratosis of hair follicle, hypochondriasis, and hematology abnormalities

Answer 467

►B Natural measles infection has been noted to improve atopic dermatitis. Atopic dermatitis typically begins in infancy, with ~50% in the first year of life and an additional 30% between 1 and 5 years. Most children with AD eventually develop allergic rhinitis or asthma later in childhood. Many outgrow AD as respiratory allergy develops. S. aureus is found in over 90% of AD skin lesions. Decreased expression of innate antimicrobial peptides, such as human beta defensin and cathelicidins, such as LL 37, may explain the increased susceptibility to colonization and skin infection with S. aureus in patients with atopic dermatitis.

Answer 468

►A The child pictured has atopic dermatitis. Features associated with atopic dermatitis include pityriasis alba, white dermatographism and delayed blanch reponse, anterior subcapsular cataracts, and keratoconus.

Answer 469

►C Sweet's syndrome is also associated with acute myelogenous leukemia, colony-stimulating factor, all-trans retinoic acid and tetracyclines. Flu-like symptoms, high-grade fever, malaise and peripheral neutrophils accompany the cutaneous lesions. Steroids and dapsone are the treatments of choice.

Answer 470

►A Sarcoidosis is a non-infectious granulomatous disorder of unclear etiology. It has a predilection for the lungs (90%), lymph nodes (75-90%), eyes (25%), nasal mucosa (20%), bone marrow (2540%), and liver (15-40%).

Answer 471

►B Dissecting cellulitis is part of the follicular occlusion tetrad which also includes acne conglobata, hidradenitis suppurtiva, and pilonidal cysts.

Answer 472

►D Neonatal lupus involves annular scaling macules and plaques appearing within the first few months of life in babies born to mothers with LE or other autoimmune connective tissue diseases. In addition to the rash, babies display congenital heart block, hepatic disease, and thrombocytopenia.

Answer 473

►C This patient has dermatomyositis. Anti-Mi-2 antibodies in DM correlate with the presence of a shawl sign, cuticular changes, and good prognosis. Anti-Jo-1 antibodies correlate with pulmonary fibrosis, Raynaud's, and polyarthritis. Anti-SRP antibodies correlate with cardiac disease and poor prognosis. Anti-Ku antibodies correlate with sclerodermatomyositis. Anti-La antibodies correlate with Sjogren's syndrome.

Answer 474

►A The changes described above are similar to findings of scleroderma. Patients receiving bleomycin can develop a reversible syndrome similar to scleroderma. The other four listed options are related to drug-induced lupus erythematosus and are not linked to scleroderma-like syndromes.

Answer 475

►B Primary systemic amyloidosis, often myeloma associated, is characterized by the deposition of amyloid fibrils derived from immunoglobulin light chains. 40% of patients manifest with cutaneous findings including petechiae, purpura, waxy translucent papules on the eyelids, diffuse alopecia, scleroderma and nail dystrophy.

Answer 476

►B Cryosurgery is the application of low temperature to produce local tissue destruction. -20- -30 degrees C is needed to destroy keratinocytes. -4- -7 degrees are needed to destroy melanocytes. Temperatures of -50 are recommended to treat malignant lesions.

Answer 477

►D This slide shows perioral dermatitis, characterized by small, erythematous papules in a perioral, often periorificial, distribution. Topical corticosteroids can exacerbate or trigger perioral dermatitis. Note the absence of comedones, which distinguishes rosacea and its subsets from acne.

Answer 478

►D The patient presents with classic findings of the oral-ocular-genital syndrome seen in the setting of vitamin B2 (riboflavin) deficiency. This deficiency occurs most often in alcoholics. It characteristically presents with angular cheilitis, atrophic glossitis (magenta), a seborrheic-like dermatitis around the nose, genital dermatitis (scrotal dermatitis sparing the mi dline and extending to the thighs), photophobia, and blepharitis.

Answer 479

►C Expression of keratins K6 and K16 is upregulated in hyperprolferative psoriasis plaques.

Answer 480

►A The most common location for folliculotropic CTCL is head/neck. It can present as a infiltrative plaque, acneiform lesions or keratosis pilaris like lesions and is usually very pruritic. The treatment of choice is PUVA.

Answer 481

►A Pseudoparalysis of Parrot is a sign of early congenital syphilis. Early congenital syphilis occurs before 2 years of age, whereas late congenital syphilis generally occurs after 2 years of age. Higoumenakis sign, clutton joints, mulberry molars, and saddle nose deformity are all signs of late congenital syphilis.

Answer 482

►E Acne conglobata is a sever variant of acne vulgaris characterized by large, often multiple comedones, abscesses with sinus formation and inflammatory nodules. Hidradenitis suppurativa is a chronic condition characterized by swollen, painful, inflamed lesions in the axillae, groin, and other parts of the body that contain apocrine glands. Dissecting cellulites of the scalp, also known as perifolliculitis capitis abscedens et Suffodiens of Hoffman, consists of deep inflammatory boggy nodules +/- sinus tracts on the occipital scalp, most commonly in African American males.

Answer 483

►A Bradykinins are responsible for angiotensin converting enzyme inhibitor induced angioedema.

Answer 484

►B The syndrome described is that of acne fulminans. Osteolytic bone lesions may accompany this syndrome, most commonly of the clavicle. Dimpling above the 5th MCP is a feature of Albright‘s Hereditary Osteodystrophy. Osteopoikilosis is a feature of Buschke-Ollendorf syndrome. Stippled epiphyses occurs in chondrodysplasia punctata. Jaw cysts are a feature of Gorlin‘s syndrome.

Answer 485

►A MAGIC syndrome is a combination of Behçets disease and relapsing polychondritis. Patients show mouth and genital ulcers with inflamed cartilage.

Answer 486

►B The most common benign neoplasm is Trichoblastoma.

Answer 487

►A Sclerosing lymphangitis is a self-limited, cord-like structure on the penile shaft, which typically occurs 24-48 hours after vigorous sexual intercourse.

Answer 488

►A Heerfordt's syndrome is the name given to sarcoidosis presenting with uveitis, facial nerve palsy, fever and parotid gland swelling. Lofgren's syndrome is an acute presentation of sarcoidosis that presents wth fever, arthritis, erythema nodosum and hilar adenopathy. Darier-Roussy disease is sarcoidosis presenting as painless firm subcutaneous nodules. Lupus pernio refers to sarcoidosis presenting as papulonodules and plaques involving the nose (especially the alar rim), ears and cheeks. Schaumann syndrome was a distractor as Shaumann bodies are seen in sarcoidosis on histopathology.

Answer 489

►A Heerfordt-Waldenstrom Syndrome is a form of sarcoidosis consisting of fever, parotid enlargement, facial nerve palsy, and anterior uveitis. Periungual telangiectasias are commonly seen in collage vascular diseases. Nail pits are commonly seen in psoriasis and alopecia areata. Sarcoidosis involvement of the scalp often results in a scarring alopecia. Condyloma lata is seen in secondary syphilis.

Answer 490

►C Perls stain stains hemosiderin (iron) blue. Fontana Masson stains melanin black. Minocycline hyperpigmentation often shows positive staining for both iron and melanin.

Answer 491

►D Leukokeratosis nicotina palati, also called smokerï¿½s palate, is a complication of tobacco use, especially pipe smokers. Lesions develop as a result of inflamed minor salivary glands and manifest as uniform keratosis of the hard palate with multiple red, umbilicated papules.

Answer 492

►E Disseminated superficial actinic porokeratosis (DSAP) is a disorder characterized by numerous keratotic macules and papules often localized in a photodistribution. Clinically, they are distinguished by a rim of keratotic scale and often may have an atropic appearance centrally. Histologically, they often display a lichenoid infiltrate flanked by characteristic cornoid lamella (inward-bending tiers of parakeratosis with underlying hypogranulosis and dyskeratosis). Recently, mutations in SART3 (squamous cell carcinoma antigen recognized by T -cells 3) have been implicated in a Taiwainese patient cohort affected by DSAP. Of note, while all varieties of porokeratoses have the potential for malignant degeneration and development of squamous cell carcinoma, lesions of DSAP have the lowest risk.

Answer 493

►C Xanthoma disseminatum is a rare mucocutaneous disease of discrete yellowish-red to brown papules in the axillary and inguinal folds. Patient are generally normolipidemic. Involvement of the pituitary gland may occur, leading to diabetes insipidus.

Answer 494

►D The "flag sign" is a striking physical finding most readily seen in long and dark hair. Hair that has grown during periods of inadequate nutrition is pale so alternating bands of light and dark can be seen along a single strand (reflecting periods of adequate and inadequate nutrition). The "flag sign" can be seen with other nutritional disorders as well.

Answer 495

►D Answer D. Pilomatricomas are benign tumors that are derived from hair matrix cells. This tumor presents more frequently in children as a solitary asymptomatic purple red papule or nodule usually on the head and neck. Mutations in beta catenin is associated with the development of these tumors. Multiple pilomatricomas may be a marker for the development of myotonic dystrophy. This rare disorder is AD, and is associated with difficulty relaxing muscles after contraction. In a review of patients with this disorder, patients tended to have mulitple pilomatricomas. Pilomatricomas can also be found with Turner syndrome, Rubinstein-Taybi, and Churg Strauss syndromes.

Answer 496

►E Spindle cell lipoma is a solitary benign tumor seen in adult male patients, and is most often located on the posterior shoulder and neck regions.The tumor histologically consists of mature collagen, adpose tissue, spindle cells, and mast cells. Treatment is with local excision.

Answer 497

►E Favre-Racouchot syndrome is a condition that generally affects elderly white males with history of chronic sun exposure and smoking. It is characterized by multiple open comedones on the temples and forehead with a background of actinincally damaged skin.

Answer 498

►E Chronic idiopathic urticaria is defined by the presence of urticaria of unknown etiology lasting greater than 6 weeks. Patient with chronic idiopathic urticaria should avoid aspirin as it aggravates urticaria in about 30% of patients.

Answer 499

►E Spironolactone is a steroid molecule. It acts as an anti-androgen by blocking the androgen receptor and inhibiting androgen synthesis.

Answer 500

►D The active ingredient in Dovonex is easily inactivated, particularly by acidic compounds like salicylic acid and lactic acid.

Answer 501

►A Intermediate filaments are composed of keratins, vimentin, desmin, peripherin, neurofilaments, nuclear laminins, and nestin. These are part of cytoskeletal elements.

Answer 502

►E Lofgren syndrome is a form of acute, self resolving, sarcoidosis manifested with hilar adenopathy, fever, migrating polyarthritis, acute iritis, and erythema nodosum. In general these patients do not have other manifestations of cutaneous sarcoidosis. Papules along the nasal rim are suggestive of Lupus Pernio sarcoidosis and suggest a worse prognosis with bronchial involvement.

Answer 503

D Patients that are diagnosed with necrolytic migratory erythema have a 75% chance of having metastatic disease by the time of diagnosis. Lesions have a circinate pattern due to peripheral spread. Patient experience adult onset diabetes, glucose intolerance and weight loss.

Answer 504

►C This patient has juvenile dermatomyositis which can feature a heliotrope rash, periungual telangiectasias, psoriasiform dermatitis, calcinosis and lipodystrophy. Thrombospondin-1 is a mediator of angiogeneis that is increased in patients with juvenile dermatomyositis. Systemic sclerosis can demonstrate antibodies to fibrillarin.

Answer 505

►A Woronoff ring is an area of blanching around psoriatic plaques secondary to decrease in prostagladin, PGE2. Koebnerization is seen in 20% of patients. Auspitz sign is bleeding upon removal of scale.

Answer 506

►C Lichen amyloidosis is a pruritic eruption that often occurs in areas of chronic rubbing. Clinically, it has a rippled appearance. Keratin derived amyloid is the type deposited in the skin.

Answer 507

►E Patients with dermatomyositis (DM) may have cancer that precedes, occurs simultaneously as, or follows the diagnosis of DM. Ovarian cancer is overrepresented in this population.

Answer 508

►D Degos disease is an obliterative arteritis clinically presenting with pink-red papules that later develop atrophic, porcelain-white centers. Death is usually secondary to Infarction of the bowel and perforation. Less commonly, death results from cerebral infarction.

Answer 509

►A The Q switched Nd:YAG can also be used to treat tan pigment. QS alexandrite and QS ruby can remove green pigment. QS Nd:YAG is a good choice for patients with darker skin color.

Answer 510

►A A patient with acanthosis nigricans can also have florid oral papillomatosis. The most commonly seen cancer is gastric carcinoma. Other cancers associated are lung, br east, uterine, ovarian, lymphoma and mycosis fungoides. It is associated with an increase in growth hormone.

Answer 511

►A Gluten-sensitive enteropathy or Celiac disease is demonstrated on small bowel biopsy of all patients with dermatitis herpetiformis. However, most of these patients do not demonstrate symptoms of gastrointestinal disease. Dermatitis herpetiformis (DH) is not associated with inflammatory bowel disease, autoimmune hepatitis, herpes labialis, or rheumatoid arthritis.

Answer 512

►E Pyoderma gangrenosum is an destructive, inflammatory disease of the skin characterized by a painful nodule or pustule that later forms a progressively enlarging ulcer. On pathology, there is a neutrophilic infiltrate with leukocytoclasia. Diseases that have been associated with pyoderma gangrenosum include inflammatory bowel disease, arthritis, an IgA monoclonal gammopathy (primarily), and myelodysplasia.

Answer 513

►D Vitiligo is an acquired disease in which there is total loss of pigment. The central process in vitiligo is the destruction of melanocytes. With silver stains or dopa reaction, well established lesions of vitiligo are completely devoid of melanocytes.

Answer 514

►B The patient has Reiter's syndrome. Reiter's syndrome is a chronic in flammatory disease similar to psoriasis with psoriatic arthritis, and is though to be a variant form. The classic triad includes urethritis, conjunctivitis, and arthritis. Few patients present with the classic triad, and thus the syndrome can be diagnosed with peripheral arthritis for greater then one month duration and rarely occurs in women. Skin findings include keratoderma blennorrhagicum and circinate balanitis (in men), as well as oral erosions, sever stomatitis, and nail changes. Chronic deforming arthritis occurs in 20% of patients. Treatments include topical steroids, NSAIDs, methotrexate, acitretin, cyclosporin, and TNF-inhibiting biologics such as etanercept.

Answer 515

►A This patient has Lofgren‘s syndrome, a variant of sarcoidosis, with erythema nodosum, hilar adenopathy, fever, and arthritis. Rheumatoid arthritis, psoriatic arthritis

Answer 516

►A Heerfordt syndrome is also known as uveoparotid fever. It is characterized by fever, uveitis, which may precede parotid enlargement and facial nerve palsy.

Answer 517

►A Palmoplantar hyperkeratosis is a cardinal feature of juvenile pityriasis rubra pilaris. It occurs in the age of 5-10 years of age and can follow after an infection.

Answer 518

►A Patients with carcinoid syndrome will develop telangiectasia, pellagra like or sclerodermoid like eruption. The neoplasm originated in the GI tract and 5-hydroxyindole-acetic acid (5-HIAA) levels would be elevated. Surgical treatment is the therapy.

Answer 519

►A Types of angioedema mediated through kinin release include hereditary angioedema, acquired C1 inhibitor deficiency, and ACE inhibitor associated angioedema. These conditions are not associated with concurrent urticaria.

Answer 520

►C Eosinophilic pustular folliculitis is a non-infectious eosinophilic infiltrate of the hair follicle. It is characterized by intense pruritus. The 3 variants of eosinophilic folliculitis include classic eosinophilic pustular folliculitis, immunosuppression-associated (mostly HIV-related), and infancy- associated eosinophilic folliculitis. The classic type (Ofuji′s disease) is more common in Japanese men. The male-to-female ratio of eosinophilic folliculitis is 5:1. It is associated with immunosuppresion and has been classified as an AIDS defining illness. Eosinophilic pustular folliculitis should be viewed as a possible cutaneous sign of immunosuppression.

Answer 521

►D Several drugs have been incriminated as inducers of psoriasis, in particular and most notably lithium, beta-blockers, antimalarials, and interferon. More recent additions include terbinafine, calcium channel blockers, captopril, glyburide, and lipid-lowering drugs such as gemfibrozil

Answer 522

►A Cutis marmorata telangietatica is seen in newborn infants when exposed to cold weather with a reticulated pattern. It represents a vasomotor response and decreases with age.

Answer 523

►B Poikilodermatous changes on the lateral thigh represent the "holster sign"Â€Â* seen in dermatomyositis. The poikilodermatous patches seen in dermatomyositis are often symmetric macular violaceous erythemas with dyspigmentation. Other characteristic poikilodermatous manifestations of dermatomyositis include the "V-sign" on the anterior neck and upper chest and the "shawl sign"Â€Â™ on the posterior neck, upper back, and the posterior shoulders. Lesions of Mycosis Fungoides typically have some component of epidermal atrophy.

Answer 524

►E Theoretically, decreased enterohepatic absorption of hormones due to altered gut flora could decrease contraceptive efficacy. However, this theory has not been borne out in studies. Only rifampin, which is a potent hepatic microenzyme inducer, has been definitively shown to reduce contraceptive efficacy.

Answer 525

►A Mast cell mediators can be grouped into two classes: preformed and newly formed. Preformed mediators include tryptase, histamine, serotonin, and heparin. Newly formed mediators include prostaglandin D2, leukotriene C4, and platelet activating factor.

Answer 526

►A Behcet's disease is associated with HLA-B51. HLA-Cw6 is the HLA type most closely associated with psoriasis. HLA-B13 and HLA-B17 are also both associated with psoriasis; HLAB17 is assiated with earlier onset and more serious psoriasis.

Answer 527

►C Lymphomatoid papulosis Lymphomatoid papulosis (LyP; Macaulay disease) is a chronic lymphoproliferative disease of the skin characterized by recurrent crops of papules that may ulcerate. The papules heal spontaneously over a period of 1-2 months and may leave a depressed scars. The T-cells in this disorder typically stain positively for CD 30.

Answer 528

►C Mucocutaneous disease is most commonly caused by New World species, although Old World L aethiopica has been reported to cause this syndrome. Infection by Leishmania viannia braziliensis may lead to mucosal involvement in up to 10% of infections depending on the region in which it was acquired. Initial infection is characterized by a persistent cutaneous lesion that eventually heals, although as many as 30% of patients report no prior evidence of leishmaniasis. Several years later, oral and respiratory mucosal involvement occurs, causing inflammation and mutilation of the nose, mouth, oropharynx, and trachea.

Answer 529

►A Macular amyloid is a form of keratin derived amyloid, which is typically located between the shoulder blades. Many stains are used to identify amyloid in the skin including Congo red, cotton dyes, crystal violet, PAS and thioflavin T.

Answer 530

►A The triceps muscle is involved early in the disease; therefore, it has the highest yield in a muscle biopsy looking for the changes seen in dermatomyositis. Surgeons have traditionally biopsied the deltoid mulscle, but it is not involved until late in the disease.

Answer 531

►E Oral lichen planus is often found in patients with hepatitis C. The association between cutaneous lichen planus and hepatitis C is not clear

Answer 532

►C The patient likely has dialysis-related amyloidosis. In this condition, beta 2-microglobulin is the protein component altered by uremia. Carpal tunnel syndrome, bone cysts, and spondyloarthropathy commonly result. Amyloid P component and ground substance are found in all forms of amyloidosis; the protein-derived amyloid fibers differ among the various forms.

Answer 533

►D Hisrutism describes excessive terminal hair growth in areas on women that are normally found only in post-pubescent males (such as beard, chest, inner thigh). Hypertrichosis refers to excessive hair density or length. Hirsutism is under the influence of androgen stimulation. Testosterone and DHEA-S can be used to detect excessive adrenal or ovarian androgen production. Other clinical manifestations of androgen excess in women are acne and virilization.

Answer 534

►E Anti-Sm is diagnostic of SLE and not reported in patients with other connective tissue diseases. It is found in 15-40% of patients with SLE. Most patients with anti-Sm also have antibodies to U1RNP, but the converse is not true. anti-U1RNP is found in 100% of patients with MCTD and in 30% of patients with SLE (the majority of patients with positive U1RNP have SLE rather than MCTD). Anti-dsDNA correlates with renal involvement in SLE. Anti-Ro and anti-La antibodies are found in LE and Sjogren's, and strongly associated with photosensitivity.

Answer 535

►E All of the above conditions are assocaited with some type of monoclonal gammopathy. Scleromyxedema is associated with an IgG-lambda monoclonal gammopathy. Scleredema and NXG are most often associated with IgG-kappa, and both POEMS syndrome and EED are usually associated with IgA monoclonal gammopathies.

Answer 536

►A Erythema nodosum can be triggered b y several medical conditions, including drugs, IBD, infections (strep, hepatitis, TB, fungus), sarcoidosis, and malginancy. Thyroid disease is not a known trigger for erythema nodosum

Answer 537

►D The boiling point of liquid nitrogen is -195.8. Nitrous oxide is -89.5. Carbon dioxide is -78.5. Dichlorotrifluoroethane (Freon 114) is 3.8. Chlorodifluoromethane (Freon 22) is 40.8. Colder cryogen is needed for adequate deep destruction, making liquid nitrogen the only reliable agent of the five listed. The Freons, solid CO2 and nitrous oxide are used only for topical anesthesia and superficial destruction.

Answer 538

►E PAPA syndrome ( Pyogenic Arthritis, Pyoderma gangerosum and Acne) The image shows classic ulcerative pyoderma gangrenosum with undermined violaceous gray border. Reepithelialization occurs from the margins and the ulcer heal usually with atrophic cribriform pigmented scar. Although the classic morphologic clinical presentation of pyoderma gangrenosum is an ulceration, there are several variants (bullous, pustular, and superficial granulomatous) which differ by their clinical presentation, location, and associated diseases.

Answer 539

►A Type IIb hyperlipoproteinemia is autosomal dominant and features increased LDL and VLDL. Associated clinical findings include xanthelasma, planar, tendinous and tuberous xanthomas. Type I hyperlipoproteinemia is autosomal recessive and manifests with increased chylomicrons. Clinically, patients develop eruptive xanthomas and lipemia retinalis. Type IIa hyperlipoproteinemia is autosomal dominant. Patients have increased LDL and may develop tendinous, tuberous, planar or eruptive xanthomas in addition to xanthelasma and arcus juvenilis. Type III hyperlipoproteinemia is autosomal recessive and features increased IDL. Clinically, these patients are prone to develop palmar, planar, tendinous, tuberous and eruptive xanthomas. Type IV hyperlipoproteinemia is autosomal dominant and manifests with increased VLDL. Patients develop eruptive, tendinous and tuberous xanthomas. Type V hyperlipoproteinemia is autosomal dominant. Patients have increased chylomicrons and VLDL and may develop eruptive xanthomas and lipemia retinalis.

Answer 540

►B Esophageal dysfunction is the most common systemic finding in systemic sclerosis. A migratory polyarthritis is the first manifestation of disease in many. Pulmonary fibrosis, renal disease and cardiac conduction defects are all manifestations but not the most common.

Answer 541

►D Azeleic acid is category B in pregnancy. The others are category C (benzoyl peroxide, topical tretinoin, and bactrim) or category X (tazarotene). The categories for safety of drugs in pregnancies are as follows: Category A: controlled studies in humans show no risk to fetus. Category B: controlled human studies show no risk to fetus but may show risk to animals, or no risk in animal studies but no human studies conducted. Category C: risk to human fetus cannot be ruled out, studies are lacking; animal studies equivocal. Category D: controlled studies show risk to human fetus, benefits may sometimes outweigh risk. Cateogory X: contraindicated in pregnancy.

Answer 542

►B Lichen sclerosus et atrophicus is an inflammatory disease of the skin that most often occurs oin the genital region. Clinically, lichen sclerosus may appear as a hypopigmented, well demarcated patch with a crinkled appearance. It is often pruritic and if left untreated may result in progressive scarring. The treatment of choice is ultra potent topical steroids.

Answer 543

►C Anti-U1RNP is found in 100% of patients with MCTD and in 30% of patients with SLE; patients with SLE and anti-U1RNP also have other positive serologies. The majority of patients with positive U1RNP have SLE rather than MCTD. The presence of U1RNP autoantibodies is associated with sclerodactyly, Raynaud's, and esophageal dysmotility.

Answer 544

►A Primary systemic amyloidosis involves the deposition of protein AL, which is derived from Ig light chains. AL is also found in nodular amyloidosis produced by a plasmacytoma. 40% of patients with primary systemic amyloidosis have skin involvement.

Answer 545

►D Zinc deficiency can be seen in premature or term infants being weaned off breast milk, which is usually high in zinc content, as well as in parenteral nutrition use, alcoholism because of poor nutritional intake, malabsorption, IBD, diets high in grains containing phytate which binds zinc, and metabolic stress

Answer 546

►D Necrobiosis is seen in GA and NLD as well as rheumatoid nodules, interstitial granulomatous dermatitis and palisading neutrophilic and granulomatous dermatitis. Mucin is also present in GA. NLD shows giant cells, extracellular lipid, and vascular changes.

Answer 547

►E Acne or acneiform erruptions can be caused or exacerbated by corticosteroids, oral contraceptives, androgens, ACTH, lithium, phenytoin, INH, and haloperidol

Answer 548

►A Capsaicin is an antipruritic and analgesic that works by depleting substance P. Repeated use leads to decreased heat, pain, and itch sensations. Doxepin is a topical tricyclic antidepressant. Diphenhydramine is an antihistamine. Pramoxine is a topical anesthetic. Gabapentin, commonly used to treat notalgia paresthetica, has an unknown mechanism of action.

Answer 549

►E Lithium, beta-blockers, interferons, ACE inhibitors, and granulocyte-colony stimulating factors have all been known to cause exacerbation of drug induced psoriasis.

Answer 550

►B All forms of amyloidosis contain amyloid P component and ground substance. The proteinderived amyloid fibers differ among various forms of amyloid. Protein AL is found in primary systemic amyloidosis. Protein AA is found in secondary systemic amyloidosis. Secondary systemic amyloid (AA) loses its birefringence after treatment with potassium permanganate, but amyloid in primary and localized forms do not.

Answer 551

►A Retinoid activity is mediated by retinoid receptors. Two groups exist: RA receptors (RAR) and RX receptors (RXR). Each has three receptor subtypes: alpha, beta, gamma. RAR-gamma is the most important mediator of retinoid activity in the skin.

Answer 552

►A Alopecia is the only cutaneous manifestation of vitamin D deficiency. Edema is the only cutaneous manifestation of vitamine B1 (thiamine) deficiency (Beriberi).Follicular hyperkeratosis is seen in hypervitaminosis A. Angular cheilitis is seen in vitamin B2 (riboflavin), B6 (pyridoxine), folic acid, niacin (vitamin B3 or nicotinic acid), biotin, zinc, and iron deficiency. Atrophic glossitis is observed in vitamin B2 (riboflavin), vitamin B6 (pyridoxine), vitamin B12, folic acid, and iron deficiency (among others).

Answer 553

►B A patient whose acne fails to respond to conventional therapy, whose acne flares cyclically, with hirsutism, alopecia, or irregular menses warrants an endocrine work-up, including free and total testosterone, LH, FSH, and DHEA-S. Although rare, very high levels of DHEA-S may suggest an adrenal adrogen-secreting tumor.

Answer 554

►B Paraphenylenediamine (PPD) may cross react with a number of substances, including azo dyes, benzocaine, procaine, sulfa drugs, and para-aminobenzoic acid (PABA). PPD is often found in dark hair dyes and in modified henna tattoos. Fragrance, nickel, lidocaine, and adhesive do not show cross-reaction with PPD.

Answer 555

Correct choice: D. IL-10 Explanation: This patient is presenting with atopic dermatitis which is a Th2 mediated disease. IL-10, along with IL-4, 5, and 13, are TH-2 cytokines. Dupilumab is a monoclonal antibody that blocks the IL-4R alpha subunit, which lowers IL-4 and IL-13, and is approved for moderate to severe atopic dermatitis. IL-2, IL-12, TNF-b, and IFN-y are all associated with a TH1 response. Reference: Odom RB. Andrews’ Diseases of the Skin, 9th ed. Philadelphia: WB Saunders; 2000.

Answer 556

Correct choice: E. All of the above Explanation: Reactivation of latent viruses such as HHV-6, HHV-7, EBV, and CMV can increase the risk of drug hypersensitivity. Drug hypersensitivity syndrome is characterized by fever, skin eruption and internal organ involvement.

Answer 557

Correct choice: E. All of the above Explanation: All of the molecules have been implicated in playing an important pathogenic role in development of SJS/TEN.

General Dermatology Flashcards

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