CLINICAL DERM Flashcards

Question

Necrobiotic xanthogranuloma is most commonly associated with which of the following: 1 IgA monoclonal gammopathy 2 IgM monoclonal gammopathy 3 IgG lambda monoclonal gammopathy 4 IgG kappa monoclonal gammopathy 5 IgG/IgA polyclonal gammopathy

Answer 1

IgG kappa monoclonal gammopathy 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 2

HBD-2 and LL 37 are significantly decreased in atopic dermatitis lesions compared to psoriasis lesions 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. IL-4 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 3

IgG lambda 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 4

Topical corticosteroids 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 5

Clonidine 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 6

Erythromycin 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 7

Inhibition of RNA-dependent protein synthesis by binding to the 50s ribosomal subunit Azithromycin is a macrolide antibiotic. It binds the bacterial 50s ribosomal subunit and inhibits RNA-dependent protein synthesis.

Answer 8

Lacrimal gland enlargement 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 9

Ducas and Kapetanakis pigmented purpura 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 10

Thrombocytopenia 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 11

IgM staining of colloid bodies Colloid bodies are apoptotic cell remnants in the papillary dermis. In lichen planus, the colloid bodies will often stain positively with IgM.

Answer 12

Parakeratosis This slide shows lichen planus of the buccal mucosa. Mucosal surfaces lack a granular layer. Parakeratosis, rather than compact orthokeratosis, is prominent.

Answer 13

Lungs 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 (25-40%), and liver (15-40%).

Answer 14

anti-Sm 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 15

Captopril 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 16

B. burgdorferi 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 morphea. mRNA specific for Borrelia has been found in some lesions of morphea. This association however is controversial.

Answer 17

Anti- Jo-1 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 18

RAR-gamma 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 19

Vitamin B12 Vitamin B12 can cause acneiform eruptions.

Answer 20

Linea nigra 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 21

High potency topical steroid The condition depicted is lichen sclerosus et atrophicus which most commonly affects the genital region. High potency topical steroids are the treatment of choice.

Answer 22

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

Answer 23

Anti-centromere 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 24

Isotretinoin 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 25

AK 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 26

``` Adiposis dolorosa 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 27

A chronic deforming arthritis occurs in 20% The patient has Reiter's syndrome. Reiter's syndrome is a chronic inflammatory 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 28

captopril 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 29

Type VII collagen 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. These interactions promote epidermal adhesion and also play a key role in a number of dermatologic diseases. Important molecular interactions include those between: (1) plakin family members, BPAG1 and plectin, with keratin intermediate filaments; (2) the former with BPAG2 and integrin α6β4 (specifically, the large cytoplasmic domain of integrin subunit β4); (3) the cytoplasmic domains of BPAG2 and integrin subunit β4; (4) the extracellular domains of BPAG2 and integrin subunit α6 as well as laminin 5; (5) integrin α6β4 in hemidesmosomes and laminin 5 in the lamina densa; (6) laminin 5 and type VII collagen; (7) type VII collagen with type IV collagen, fibronectin, and type I collagen in the sublamina densa region.

Answer 30

A decreased level of serum C1q 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 31

5-10% Nodular amyloidosis is a rare type of primary cutaneous amyloidosis made up of AL protein. The risk for systemic involvement is 7%.

Answer 32

Anti-Mi-2 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 33

anti-155/140 **_Anti-155/140**_ has been associated with an increased risk of _**malignancy_** in dermatomyositis patients. **_Anti-SRP**_ is associated with _**fulminant dermatomyositis/polymyositis**_ and _**cardia_**c 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 34

1 month 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 35

``` Polyclonal IgM and polyclonal IgG 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 36

HLA-B27 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 37

Lung cancer 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 38

Fibrodysplasia ossificans progressiva 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. Plate-like 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 39

Rifampin 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 40

Îbeta-hemolytic streptococcal infection 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 41

Non-Hodgkin lymphoma 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 42

Granulocyte colony stimulating factor 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. Edema of the upper dermis is marked. There is a primarily neurtophilic perivascular infiltrate in the mid dermis with a few mononuclear cells and eosinophils. The capillaries show endothelial swelling with some leukocytoclasia but no frank vasculitis. symmetric edematous violaceous plaques some with central pustules

Answer 43

Squamous cell carcinoma Acrokeratosis paraneoplastica, also known as Bazex’s 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. A 70 year old man developed a psoriasiform dermatitis of the fingers, toes and ears (helix). He had a destructive nail dystrophy. A few weeks later the patient also developed cervical adenopathy. Otolaryngologic investigations revealed a layngeal squamous cell carcinoma.The Bazex syndrome or acrokeratosis paraneoplastica is a rare dermatosos associated with carcinomas of the upper aerodigestive tract with possible lymph node mestastases. The cutaneous lesions occuring in the syndrome are non metastatic skin involvment. The psoriasiform lesions often precede the diagnosis of the associated malignancy, or occur simultaneously. The reapparance of skin lesions may signal a recurrence of the tumor. Treatment consists in treatment of the underlying cancer. In some cases, acitretin or oral psoralen phototherapy could be prescribed. Description symmetric erythematous scaly patches with yellowing and scaling of the nails

Answer 44

Insulin resistance 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 45

IL-1 IL-1 is a pro-inflammatory cytokine that has been associated with follicular plugging and microcomedo formation.

Answer 46

Leishmania brasiliensis 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. This 68-year-old woman developed destruction of the nose, soft palate and central hard palate resulting in difficulty with breathing following scarring from leishmaniasis. Description destruction of the nose and scarring of the hard palate

Answer 47

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

Answer 48

Systemic lupus erythematosus 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. A healthy 9-year-old girl with systemic lupus erythematosys in remission developed an acute flare of cutaneous lesions after intensive early spring sun exposure. Although she had no evidence of systemic disease, she also complained of painful ulcers on the palate, tongue,and lips. Description photodistributed symmetric red scaly papules and patches

Answer 49

Serum free and total testosterone, LH, FSH, DHEA-S 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. This 31-year-old woman had a 2 year history of male pattern growth of terminal hair on her face, chest, and areolae. Hormonal assays showed increased levels of total testosterone and dehydroepiandrosterone and the abdominal ultrasound revealed changes typical of polycystic ovaries. She was treated with long term oral birth control pills (Diane 35). Description coarse dark male pattern terminal hair

Answer 50

Von Kossa 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. A 27 year old woman with chronic pruritis associated with Alagille syndrome since early childhood developed uniform pruritic lichenoid papules on her shins. Histopathology showed typical changes of lichen amyloidosis. Description uniform 2 mm flat topped skin colored to hypopigmented papules

Answer 51

Sensorimotor neuropathy 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. 56 yo female presents with chronic urticaria, intermittent fever, lymphadenopathy, hip pain. Elevated sedimentation rate and IgM kappa monoclonal gammopathy. Thought to be due to chronic activation of IL-1 alpa

Answer 52

Favre-Racouchot 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 53

Ethyl cyanoacrylate 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 54

IgG lambda An abnormal paraproteinemia is found in 90% of cases of scleromyxedema, usually IgG lamda. This 45-year-old woman developed progressive acral skin induration. She had no evidence of Raynaud phenomenon, calcinosis cutis, esophageal reflux, or telangiectasias. A biopsy showed changes typical of scleromyxedema, and urinary and serum protein electrophoresis were normal. Description diffuse induration with a pebbly texture and fine scale

Answer 55

``` PAPA syndrome 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. ``` SAPHO A 37-year-old woman with a history of palmoplantar pustulosis presented with severe chest pain. The patient was first diagnosed with palmoplantar pustulosis at the age of 36 and soon afterwards suffered from upper chest pain. Although the pain and skin lesion had been relieved for three years after excision of the left first sternocostal articulation, she experienced subsequent relapse of sternal pain and palmoplantar pustulosis. Medication for rheumatoid arthritis, such as prednisolone, bucillamine, and methotrexate, or antibiotic therapy failed to ameliorate the symptoms. At the age of 45, she underwent palatine tonsillectomy with resolution of symptoms. At present, the patient has neither palmoplantar skin lesions nor sternal pain. Description symmetric thick scaly red acral plaques studded with pustules

Answer 56

Myotonic dystrophy Answer D. Pilomatricomas are benign tumors that are derived from hair matrix cells. This tumor presents more frequently in children as a solitary asympotmatic 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 57

Sipple syndrome The image depicts a case of macular amyloid with the typical reticulated or rippled hyperpigmentation. MAcular amyloid may be found in Sipple syndrome, also called MEN IIa. Other features of MEN IIa include pheochromocytoma, parathyroid cancer and pancreatic cancer.

Answer 58

Natural measles infection has been noted to improve atopic dermatitis 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 59

Sarcoid 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 60

Alcoholism 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 61

Corn 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 62

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

Answer 63

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

Answer 64

ssDNA 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 65

Steroid inhibitor of androgen receptor and androgen biosynthesis Spironolactone is a steroid molecule. It acts as an anti-androgen by blocking the androgen receptor and inhibiting androgen synthesis.

Answer 66

Sarcoidosis 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 67

Psoriasis This slide depicts nail changes consistent with psoriasis, including onycholysis, subungual debris and pits. Pits in alopecia areata are usually more regularly spaced than in psoriasis. Fungal culture of nail clipping for histologic evidence of onychomycosis is warranted. The main feature of tetracycline-induced photo-onycholysis is onycholysis, rather than chronic nail bed and matrix involvement with psoriasis.

Answer 68

20% 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 69

kwashiorkor 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 70

Represents inflamed minor salivary glands Leukokeratosis nicotina palati, also called smoker's 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 71

low C2 and C4 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 72

Keratin 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 73

Dermatomyositis Poikilodermatous changes on the lateral thigh represent the "holster sign" 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" on the posterior neck, upper back, and the posterior shoulders. Lesions of Mycosis Fungoides typically have some component of epidermal atrophy.

Answer 74

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

Answer 75

beta 2-microglobulin is likely causative 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 76

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

Answer 77

Facial edema 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 78

Relapsing polychondritis Relapsing polychondritis is a rare disease manifested by recurring inflammation of cartilaginous 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 79

Sensitivity: 60 Specificity: 80 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 80

Alopecia 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 81

Sweet's syndrome 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 82

Renal transplant and Cyclosporine therapy 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 83

``` Type I 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 84

Lichen sclerosus et atrophicus 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 85

Acne conglobata, hidradenitis suppurativa, dissecting cellulites of the scalp 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 86

classified as an AIDS-defining illness 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 (OfujiÃ¢ÂÂs) 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 87

Nifedipine 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 88

``` Twin concordance (identical twins) is 55% 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 89

Antibodies to transglutaminase 3 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 90

Scleroderma 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 ï¿½salt and pepperï¿½ appearance due to perifollicular depigmentation.

Answer 91

Familial Mediterranean Fever 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 92

The syndrome that presents with renal amyloidosis, fevers, limb pains, and deafness involves AA protein 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 93

Pyoderma gangrenosusm 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 94

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

Answer 95

Hereditary angioedema 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 96

Scleromyxedema 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 97

Atopic dermatitis 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.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.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.Atopic dermatitisAtopic dermatitisAtopic dermatitisAtopic dermatitisAtopic dermatitisAtopic dermatitisAtopic dermatitisProbiotics 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. 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. Atopic dermatitis 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. Atopic dermatitis 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 98

Micrococcus sedenditaris 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 gram-positive, 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 99

Pseudotumor cerebri 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 100

Usually occurs in young women of HLA-B27 genotype 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 101

Milroy disease Milroy disease in an autosomal dominantly inherited form of primary lymphedema. Unlike Meige disease, Milroy disease is congenital and due to a failure of the development of lymphatic vessels in utero.

Answer 102

Adapalene 0.1% gel 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 103

Drug induced pellagra 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 104

Tumor necrosis factor alpha 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.

Answer 105

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

Answer 106

Hypertrichosis is often a feature Seip-Lawrence syndrome is congenital generalized Berardinelli-Seip. These patients lack both subcutaneous fat and extracutaneous adipose tissue. The acquired form is known as Lawrence-Seip syndrome. Patients often have acanthosis nigricans, hypertrichosis, generalized hyperpigmentation, and thick, curly scalp hair. Patients have voracious appetites, perspire excessively, and may be heat-intolerant despite normal thyroid function (basic metabolic rate is elevated). Mental retardation is common.

Answer 107

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

Answer 108

Decreased C3 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 109

Activation of TLR-2 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 110

No associated disease 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 111

Topoisomerase I antibodies (formerly Scl-70) 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 112

LMNA 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 113

Lofgren's syndrome 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 114

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

Answer 115

Absence of reports of cutaneous atrophy 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 116

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

Answer 117

Obstruction of the pilosebaceous duct 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 118

anti-fodrin 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 119

Uveitis 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 120

Ophthalmologist 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 121

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

Answer 122

HLA-B27 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 123

Spread of purpura to the upper trunk 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 124

Amoxicillin 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 cross-reacting 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 125

Genitourinary 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,lumph nodes, liver, spleen and GIT are among the most common involved systems whearas genitourinary and neuroendocrine systems are least likely to be involved

Answer 126

Takayasu arteritis 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. Polyarteritis 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 127

Metroprolol 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 128

Thrombospondin-1 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 129

Amyloidosis 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 130

Relapsing polychondritis MAGIC syndrome is a combination of Beh�ets disease and relapsing polychondritis. Patients show mouth and genital ulcers with inflamed cartilage.

Answer 131

Add oral prednisone 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 132

It is often misdiagnosed as cellulitis 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 133

A positive rheumatoid factor may be seen 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 134

Ribonucleoproteins A speckled ANA pattern correlates with staining of ribonucleoproteins and is seen in Mixed Connective Tissue Disease (MCTD), Systemic Lupus Erythematosus (SLE), systemic sclerosis 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 135

Nevus achromicus 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 136

Rifampin 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 137

2,3,7,8 tetrachlorobenzodioxin exposure 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 138

Interferon-gamma 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 139

Heerfordt's syndrome 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 140

Lichen amyloidosis 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 141

Erythema nodosum The most common non-specific cutaneous manifestation of sarcoidosis is erythema nodosum. The clinical hallmark of leukocytoclastic vasculitis is palpable purpura. There are numerous causes of erythema nodosum including drugs, infection, malignancy, and idiopathic. Lichen planus can be associated with hepatitis C.

Answer 142

A-defensin 2 Human A-defensins (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 143

Face 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 144

Pseudoparalysis of Parrot 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 145

Increased staining on both Fontana Masson and Perls stain The patient has Type 2 minocycline-associated hyperpigmentation. Three types of minocycline-associated 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 146

HLA-B27 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 147

Anthralin inhibits PMNs and monocytes 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 148

Pulmonary fibrosis 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 149

Gastrointestinal 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

Answer 150

Ovarian carcinoma 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 151

The adrenals and ovaries 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 152

Lichen Planus 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 153

Macroglossia 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

Answer 154

Rectum 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 tumors 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 155

Isotretinoin 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 156

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

Answer 157

C2 and C4 are both decreased 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 158

Bradykinins Bradykinins are responsible for angiotensin converting enzyme inhibitor induced angioedema

Answer 159

Can be treated with narrow band UVB 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 160

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

Answer 161

The amyloid P component is present in all forms All forms of amyloidosis contain amyloid P component and ground substance. The protein-derived 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 162

Pits 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 163

Type XVII collagen 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 164

This condition mimics folate deficiency 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 oral-ocular-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 165

The production of serotonin, intense flushing, peptic ulcer, and lacrimation 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 serotonin, cause peptic ulcers, and produce intense, persistent flushing and lacrimation.

Answer 166

Azeleic acid 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 167

age over 40 years 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 168

The hair abnormality tends to persist with age This patient has uncombable hair syndrome, characterized by dry, spun-glass, silvery blond hair. It is also known as pili trianguli et canaliculi. Hair is normal in quantity and tends to become more manageable with time.

Answer 169

Pelvic exam 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 170

anti-SSA, anti-SSB, anti-Sm, anti-U1RNP 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 171

Blau syndrome 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 172

Azelaic acid 20% cream 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 suties show risk to human fetus, benefits may sometimes outweigh risk. Cateogory X: contraindicated in pregnancy

Answer 173

Keratin derived 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 174

Can occasionally see epidermotropic T-cells 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 175

Blue staining granules within macrophages Perls stain stains hemosiderin (iron) blue. Fontana Masson stains melanin black. Minocycline hyperpigmentation often shows positive staining for both iron and melanin

Answer 176

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

Answer 177

HLA-B51 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; HLA-B17 is assiated with earlier onset and more serious psoriasis

Answer 178

Bullae, when present, are subepidermal 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 179

Increased secretion of IL-4 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 180

Potential for bacterial resistance 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 181

Focal loss of elastic tissue 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 182

Nail changes 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 183

Asymmetric arthritis 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 184

Abrupt onset of plaques 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 185

Ear 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 186

PSTPIP1 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 proline-serine-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 187

Vitamin 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 188

Bowel infarction 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 189

Metoprolol 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 190

Vitamin B2 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 midline and extending to the thighs), photophobia, and blepharitis

Answer 191

Collagen III Keloids are dense overgrowths of fibrous tissue that usually develop after injury to the skin. Collagen III is overrepresented in these keloids

Answer 192

The sternoclavicular joint is often involved in this entity 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 193

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

Answer 194

HLA-Cw6 HLA-Cw6 is the HLA type most definitively associated with psoriasis. It carries a relative risk 9-15 times normal.

Answer 195

triceps 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 196

IgA monoclonal gammopathy and a benign course 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 197

Hepatitis C 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 198

Down syndrome 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 199

Osteolytic lesions of the clavicle 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 200

TLR-2 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 pro-inflammatory cytokines.

Answer 201

HLA-B57, HLA-Cw6, or HLA-DR7 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 202

aspirin 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 203

Activation of toll-like receptor-2 by P. acnes 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 204

Both sexes are equally affected 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 205

anti-dsDNA 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 206

C1-inh normal, C1 normal and C4 low C1 esterase inhibitor deficiency may be inherited or acquired. The genetic form is inherited in an autosomal dominant fashion. Serum C1q is low in the inherited form and therefore the defect is in the synthesis and/or function of the inhibitor. In acquired disease, serum C1q is decreased. Lymphoproliferative disease and rheumatological disease may be the cause. As far as complement levels are concerned, C2 and C4 are decreased in both forms. Androgen therapy using danazol or stanozolol are given for prevention while fresh frozen plasma or C1INH should be given in acute attacks. In cases of hereditary disease the profile is normally: C1 nl, C4 low, C2 low, C3 nl, C1-inh low or nl, C1q low, CH 50 nl or low. For acquired disease, the profile is typically: C1 low, C4 low, C2 low, C3 nl, C1-inh low or nl, C1q low, CH 50 low. C1 can distinguish between hereditary and acquired forms.

Answer 207

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

Answer 208

Anti-Jo-1 antibodies against histidyl-tRNA synthetase 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 c-ANCA 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 209

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

Answer 210

Nuclear lamina 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 211

Ulcerative colitis 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 212

Acute anterior uveitis Sarcoidosis is a systemic disease characterized by the formation of non-caseating granulomas. Organs involved include the skin, eyes, lungs, liver and spleen. The most common ocular finding is acute anterior uveitis. Other findings may include posterior uveitis, blurred vision, and excessive lacrimation.

Answer 213

Asymmetric, non-erosive polyarthritis 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 214

Taxanes 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 Î²-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 215

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

Answer 216

Vesicles may develop de novo on previously uninvolved skin 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 217

Cardiac defect 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 218

Lupus pernio 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 219

Anti-Mi-2 antibody 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. Anti-KU antibodies correlate with sclerodermatomyositis. Anti-PL7 and Anti-PL12 antibodies (anti-synthetase antibodies) also correlate with pulmonary disease.

Answer 220

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

Answer 221

CD 30 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 222

TSH Erythema nodosum can be triggered by 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 223

Azelaic acid 20% cream 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 224

20% monobenzyl ether of hydroquinone 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 225

HLA-B51 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 226

Association with underlying malignancy 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 227

Linear 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 228

``` Familial dysbetalipoproteinemia (type III) 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 obesit ```

Answer 229

Behchets disease 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 230

Diabetes The emotional and physical burden of psoriasis is easily underestimated. Rapp et al found that patients with psoriasis report reduced physical and mental functioning (�heath related quality of life�) similar to that seen in patients with cancer, hypertension, arthritis, heart disease, diabetes, and depression.

Answer 231

Pits 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 232

Ovarian Cancer 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 233

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

Answer 234

Follow a streptococcal infection This slide shows guttate psoriasis.

Answer 235

RAR-gamma 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 236

Hyperkalemia 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 237

Better oral hygiene 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 238

Liver disease 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 239

Accelerated blanch response 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 240

Congenital heart block may be the only manifestation of the disease 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 241

Beaded papules on nasal rim 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 saroidosis. Papules along the nasal rim are suggestive of Lupus Pernio sarcoidosis and suggest a worse brognosis with bronchial involvement.

Answer 242

Tetracycline 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 243

Esophageal dysfunction 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 244

Alkaline phosphatase 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 245

Vitamin D Phrynoderma or â€œtoad skinâ€ 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 B-complex vitamins and vitamins C and E, in addition to essential fatty acid deficiency.

Answer 246

Vitiligo 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 247

Sulfa drugs 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 248

Posterior shoulder 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 249

Pityriasis rubra pilaris 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 250

Streptococcus pyogenes 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 251

Produce IL-6 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 252

Acyclovir may be effective in this condition 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 253

Adrenal tumor 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 254

Dihydrotestosterone 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 255

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

Answer 256

The prescription moisturizer likely contains lactic acid. The active ingredient in Dovonex is easily inactivated, particularly by acidic compounds like salicylic acid and lactic acid.

Answer 257

Bleomycin 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 258

Aging Black hairy tongue is a common reactive condition due to the enlongation of filiform papillae. The most common etiologies include poor oral hygeine, use of oral antibiotics, HIV infection, use of tobacco, radiation, and alcohol.

Answer 259

Nifedipine 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

Bazex's syndrome 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 261

HLA-DR4 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 262

Presents with a reticulate pattern on the dorsal hands and feet 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 263

Lofgren's syndrome 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 264

Placental insufficiency 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 265

Multicentric reticulohistiocytosis 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 266

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

Answer 267

Diabetes mellitus 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. Dabski K, Winkelmann RK. Generalized granuloma annulare: clinical and laboratory findings in 100 patients. J Am Acad Dermatol. 1989;20:39-47

Answer 268

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

Answer 269

Involvement of the pituitary gland 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 270

Psoriasis arthritis This slide shows lateral onycholysis and subtle swelling of the distal interphalangeal joint (DIP), both characteristic findings of psoriasis and psoriatic arthritis.

Answer 271

``` Niacin Niacin (Vitamin B3) deficiency is associated with a photosensitive dermatitis, diarrhea and dimentia. 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 272

Amyloid is present around blood vessels 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 273

20% of both men and women with this diagnosis have at least one autoimmune disease 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 274

The lack of systemic features 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 275

anti-Jo-1 antibody 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 276

Dapsone 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 277

Anterior uveitis 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 278

Civatte 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 279

Churg-Strauss syndrome 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 280

Mandibular nerve 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 281

Idiopathic hirsutism 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 282

Corneal opacities 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 283

Mikulicz's syndrome 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 bilateral 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 284

Granulomatous uveitis 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 285

Leukemia 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 286

She is more likely to develop systemic lupus erythematosus than an adult with these lesions 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 287

Esophageal dysmotility 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 288

Pre-existing dermatoses 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 289

Albinism 2 Vitiligo 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 290

Pulse dye laser 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 291

Triglyceride 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 292

Biotin 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 293

Inhibits 14-alpha-demethylase 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 294

Appendix 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 tumors 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. Ileal tumors are the most common source of the classic carcinoid syndrome, and these tumors metastasize most frequently.

Answer 295

Ovarian cancer 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 dermatomyositis.

Answer 296

Type IIb 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 297

Doxepin 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 298

Is not androgen-dependent 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 299

Biologic Labs: 1. ANA w/ Titer & Pattern 2. CBC w/ Diff 3. CMP 4. HEP B panel 5. VDRL 6. TB (QUANTIFERON GOLD replaces PPD) 7. Vit. D

Answer 300

ICD-9: 706.1 & v67.51 FASTING: CBC w/ Diff, CMP, CHOL, TRIG, QUAL BETA HCG X2 (FEMALES)

Answer 301

FASTING CBC w/ Diff, CMP, TRIG, CHOL, QUAL BETA HCG X2 (Avoid Pregnancy 3 years after D/C of med) Diabetic patients: HbA1C

Answer 302

1. ANA, 2. Anti-dsDNA, 3. Anti-Sm, 4. Anti-U1RNP, 5. Anti-histone, 6. Anti-SS-A/Ro, 7.Anti-SS-B/La 2. Plaquenil: (Lupus 695.4) Baseline: 1. CBC w/ Diff, 2. CMP, 3. UA, 5. G6PD (Ist Time Only), 6. Yearly Ophthal exam Eye exam & CBC w/ Diff Q3mo if prolong Rx

Answer 303

Onychomycosis: 110.1 FASTING: CBC w/ Diff, CMP, PLATELET count

Answer 304

Vitiligo: 709.01 Antiparietal cell Antibodies & Antimicrosomal Antibodies

Answer 305

Thyroid: TSH & Antithyroid Antibody

Answer 306

1. Rheumatoid Arthritis 2. Methotrexate: Every 3 months: Fasting: CBC w/ Diff, CMP, UA

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