Cutaneous Manifestations of Systemic Disease Flashcards
1- The patient was recently hospitalized for a spontaneous pneumothorax. What is the underlying genetic mutation?
A. FLCN
B. CYLD
C. MSH2
D. PTEN
E. Menin
Correct choice: A. FLCN
Explanation: The figure illustrates fibrofolliculomas seen in Birt-Hogg-Dube syndrome. The syndrome features autosomal dominant inheritance, multiple fibrofolliculomas, trichodiscomas, lipomas, oral fibromas, renal cell carcinoma, medullary thyroid carcinoma, and colon cancer. Patients are at risk for pulmonary cysts and spontaneous pneumothoraces. The association mutation is FLCN (folliculin gene). The other mutations are found in Familial cylindromatosis (CYLD), Muir-Torre (MSH2), Cowden/PTEN hamartoma syndrome and Bannayan-Riley-Ruvalcaba syndrome (PTEN), and Multiple endocrine neoplasia (MEN) type I or Werner syndrome (Menin).
2- Which of the following is most commonly associated with hepatitis C infection?
A. Essential mixed cryoglobulinemia
B. Rheumatoid arthritis
C. Relapsing polychondritis
D. Wegener’s granulomatosis
E. Dermatomyositis
Correct choice: A. Essential mixed cryoglobulinemia
Explanation: Hepatitis C infections can present as urticaria or papable purpura and cryoglobulinemia. Studies in patients with mixed cryoglobulinemia syndrome found that 95% had signs of HCV infection.
3- A 35 year old man with a history of celiac disease presents with a beefy, red tongue, hyperpigmented palmar creases, and premature grey hair. Which of the following statements is correct?
A. The best therapy is riboflavin 5mg/day
B. This condition mimics folate deficiency
C. It is often associated with carcinoid tumors which divert tryptophan to serotonin
D. This condition can be caused by azithioprine, 5-FU, and isoniazid
E. Eating raw egg whites is a risk factor
Correct choice: B. This condition mimics folate deficiency
Explanation: 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.
4- Which of the following is FALSE regarding Fabry’s disease?
A. It is inherited in an X-linked recessive fashion
B. It is associated with acral parasthesias
C. It is inherited in an X-linked dominant fashion
D. It may be associated with renal failure
E. The etiology is a defect in alpha-galactosidase A
Correct choice: C. It is inherited in an X-linked dominant fashion
Explanation: Fabry’s disease is an X-linked lysosomal disorder that leads to excessive deposition of neutral glycosphingolipids in the vascular endothelium. The disorder is caused by a deficiency of alpha-galactosidase-A leading to progressive endothelial accumulation of glycosphingolipids. This accumulation accounts for the associated clinical abnormalities of skin, eye, kidney, heart, brain, and peripheral nervous system. Acroparesthesias are a frequent presenting symptom of Fabry’s disease. The primary cutaneous manifestation are angiokeratomata (angiokeratoma corporis diffusum), which are most common in a “bathing-trunk” distribution. Lens opacities and retinal and conjunctival vascular malformations may be found in the eyes. Patients may experience cardiac disease, stroke or renal failure.
5- You are called to evaluate a patient with these cutaneous findings. Once the diagnosis is confirmed, which additional test is indicated in the work-up for underlying causes?
A. Colonoscopy
B. Blood cultures
C. Hemoglobin A1C
D. Fat pad biopsy
E. Hepatitis C Antibody
Correct choice: E. Hepatitis C Antibody
Explanation: Porphyria cutanea tarda presents with vesicles and erosions with milia and scarring on sun-exposed skin, classically the dorsal hands. There is a deficiency in uroporphyrinogen decarboxylase (UD) and urine uroporphyrinogen is elevated. The most common associated conditions are Hepatitis C, HIV, iron overload states (hemochromatosis) and environmental triggers (alcohol, estrogens, polyhalogenated hydrocarbons). Treatment includes phlebotomy and low dose oral antimalarials.
6- Paraproteinemia is associated with all EXCEPT:
A. Scleromyxedema
B. Necrobiotic xanthogranuloma
C. Scleredema
D. Sclerosing panniculitis
E. Plane xanthoma
Correct choice: D. Sclerosing panniculitis
Explanation: Sclerosing panniculitis (lipodermatosclerosis) displays characteristic changes in the fat (lipomembranous change); it is not associated with paraproteinemia. Generalized plane xanthomas, scleromyxedema, necrobiotic xanthogranuloma, scleredema, erythema elevatum diutinum, xanthoma disseminatum, and pyoderma gangrenosum have all been associated with a paraproteinemia.
7- A patient with multiple deeply pigmented papules has a skin biopsy which reveals an epitheloid blue nevus. The next appropriate step is:
A. Reassure the patient and follow up as needed
B. Schedule prophylactic excision of the lesion
C. Begin a malignancy work-up
D. Refer to genetics
E. Order an echocardiogram
Correct choice: E. Order an echocardiogram
Explanation: Epithelioid blue nevi have been reported with and without association with cardiac myxomas as a component of the Carney complex (NAME/LAMB syndrome). Carney complex is an autosomal dominant disorder caused by mutations in PRKAR1A. Patients have cutaneous and atrial myxomas, blue nevi, ephelides, adrenocortical disease, and testicular tumors. The other options are not as appropriate as ordering an echocardiogram.
8- A homeless patient presents with a scaling, pustular periorifical eruption around the mouth and genitalia. What lab abnormality is associated with this condition?
A. Increased zinc level
B. Increased copper level
C. Decreased copper level
D. Decreased alkaline phosphatase level
E. Increased alkaline phosphatase level
Correct choice: D. Decreased alkaline phosphatase level
Explanation: In a homeless patient with poor nutrition, acrodermatitis enteropathica due to zinc deficiency can present with a scaling eruption of the periorificial regions. In addition to a low zinc level, levels of alkaline phosphatase, a zinc dependent enzyme, are decreased. Remaining choices are incorrect as they do not capture the lab abnormality of acrodermatitis enteropathica.
9- What is the most likely associated chronic infection?
A. Hepatitis B
B. Hepatitis C
C. HIV
D. HTLV-1
E. Mycobaterium Tuberculosis
Correct choice: B. Hepatitis C
Explanation: This is an example of necrolytic acral erythema, which is associated with chronic hepatitis C infection. Hepatitis B is associated with classic PAN, among other conditions. HIV is associated with many dermatoses. HTLV-1 is associated with mycosis fungoides. TB can be
associated with Erythema Induratum and tuberculid reactions. None of the above conditions is classically associated with necrolytic acral erythema.
10- A patient with gastric cancer develops acanthosis nigricans and a sudden eruption of numerous warty stuck-on papules on the trunk. What other finding may be seen?
A. Thickened, velvety palms with pronounced dermatoglyphics
B. Follicular spicules on the nose
C. Carpal tunnel syndrome
D. Periorbital pupura
E. Migratory thrombophlebitis
Correct choice: A. Thickened, velvety palms with pronounced dermatoglyphics
Explanation: Thickened, velvety palms with pronounced dermatoglyphics describes tripe palms (aka acanthosis palmaris). Tripe palms without acanthosis nigricans is associated with underlying lung cancer, whereas tripe palms with acanthosis nigricans is associated with underlying gastric cancer. Follicular spicules on the nose may be seen in multiple myeloma. Carpal tunnel syndrome and periorbital purpura can be seen in primary systemic amyloidosis (which can be associated with multiple myeloma). Migratory thrombophlebitis is associated with pancreatic cancer.
11- A 39 year old female has noticed a darkening area on both of her ears over the course of several decades. She denies supplement or medication intake and she does not use any topical products; otherwise she endorses a history of joint pains and stiffness. On physical exam, she has darkening of the anti-helix of both ears. What is the most likely diagnosis?
A. Argyria
B. Exogenous ochronosis
C. Relapsing polychondritis
D. Alkaptonuria
E. Dermal filler injection complication
Correct choice: D. Alkaptonuria
Explanation: The patient has alkaptonuria. The ears are a common site of involvement and show visible darkening over time. The musculoskeletal system can be involved as well. The patient does not endorse a history of any supplemental intake, thus argyria from silver toxicity is unlikely. She denies the use of any topical products, making exogenous ochronosis unlikely (and this condition is more common on the face where hydroquinone is more likely to be applied). Dermal filler injection is not correct as the patient has no history of dermal filler injection and the ear would represent an atypical site for injection of dermal filler. Relapsing polychondritis can present with musculoskeletal complaints, however, the ear and/or nose is typically inflamed and painful during episodes - our patient notices progressive darkening of the ear over time in contrast.
12- A 61-year-old woman presents with extensive erosions with hemorrhagic crusting of the lips, as well as several inflammatory erosions on the upper trunk. A peri-lesional biopsy for DIF shows deposition of C3 and IgG intercellularly and along the BMZ. Which of the following benign neoplasms is the most likely cause of this woman’s mucocutaneous lesions?
A. Thymoma
B. Uterine leiomyoma
C. Teratoma
D. Hepatic adenoma
E. Castleman’s disease
Correct choice: E. Castleman’s disease
Explanation: The stem describes the clinico-path findings seen in paraneoplastic pemphigus (PNP), which can be associated with both benign or malignant neoplasms. Among benign neoplasms, Castleman’s disease and thymoma have been reported as causes (Castleman’s disease more commonly than thymoma). Thymoma is less likely than Castleman’s disease to be the cause. The remaining answer choices are not associated with PNP.
13- A 71-year-old man presents to your clinic for a new asymptomatic, red-purple rash around his eyes. You notice that the lesions do not blanch with pressure. Which of the following is the most likely underlying malignancy?
A. Lung adenocarcinoma
B. Gastric adenocarcinoma
C. Multiple myeloma
D. Small cell lung cancer
E. Acute myelogenous leukemia (AML)
Correct choice: C. Multiple myeloma
Explanation: This patient’s presentation is concerning for periorbital “pinch” purpura, which occurs classically in primary systemic amyloidosis. Up to 25% of cases of primary systemic amyloidosis are due to multiple myeloma. Thus, this patient should undergo SPEP, UPEP, and serum immunofixation electrophoresis testing to evaluate for myeloma. The remaining listed malignancies are not typically associated with primary systemic amyloidosis.
14- Which of the following findings can be elicited during examination of these widespread skin lesions?
A. Buttonhole sign
B. Dimple sign
C. Crowe’s sign
D. Darier’s sign
E. Pseudo-Darier’s sign
Correct choice: A. Buttonhole sign
Explanation:With application of gentle pressure, a neurofibroma will easily invaginate into the subcutis, a finding known as the buttonhole sign. This question asks the examinee to identify neurofibromas by multiple soft, rubbery, skin colored to pink-tan papulonodules, and apply their knowledge of classic skin physical exam signs. When pressed on gently, a neurofibroma will invaginate into the subcutis, demonstrating the so-called buttonhole sign (choice 1). The dimple sign (choice 2) refers to the downward movement of a dermatofibroma when it is pinched. Crowe’s sign (choice 3) is axillary freckling seen in neurofibromatosis type I; it is not a feature of the neurofibromas themselves. Darier’s sign (choice 4) is local urtication with rubbing of lesions of cutaneous mastocytosis, while the pseudo-Darier’s sign (choice 5) is transient induration occurring with rubbing of a smooth muscle hamartoma.
15- this patient likely has which of the fellowing complaints in addition to the photo shown?
A- Hematuria
B- Dysphagia
C- proximal muscle weakness
D- Numerous Telangiectasia
E- Immunodeficiency
Correct choice: C-proximal muscle weakness
Explanation: Ragged cuticle is classically seen in dermatomyositis ,but can be seen in other autoimmune connective tissue diseases.
16- A patient presented with sudden onset numerous waxy brown papules on the trunk Which malignancy you should be most concerned with ?
A- lung carcinoma
B- Adenocarcinoma of GI tract
C- malignant melanoma
D- Non- Hodgkin lymphoma
E- Hodgkin lymphomas
Correct choice: B- Adenocarcinoma of GI tract
Explanation: The photo represents the sign of leser trelat which is the most commonly associated with GIT adenocarcinoma .this has also seen in lymphoma, breast ,cancer ,lung cancer.
17- A patient with history of AML was admitted for induction therapy with cytarabine and danurubicin .2 weeks later, she developed the fellowing painful rash .what is the diagnosis?
A- leukemia cutis
B- erythema elevatum diutinum
C- cystic acne
D- neutrophilic eccrine hidradenitis
E- Sarcoidosis
Correct choice: D - neutrophilic eccrine hidradenitis
Based on the primary cell type involved in pyoderma gangrenosum, which of the following treatments would be appropriate?
A. Acitretin
B. Crisaborole
C. Dapsone
D. Dupilumab
E. Apremilast
Correct choice: C. Dapsone
Explanation: Pyoderma gangrenosum (PG) is a neutrophil driven disease process. In addition to its usage as an antibiotic, dapsone is anti-inflammatory and has anti-neutrophilic properties. Topical and/or systemic dapsone preparations can be appropriate choices for the treatment of PG.
19- A biopsy from a systemically ill patient with porcelain white macules and papules on the trunk and extremities shows wedge-shaped necrosis stemming from a deep vessel. Involvement in which of the following systems most commonly is responsible for a fatal outcome?
A. Cardiac
B. Gastrointestinal
C. Renal
D. Endocrinologic
E. Neurologic
Correct choice: B. Gastrointestinal
Explanation: The correct answer is B. The question describes Degos disease (malignant atrophic papulosis). Although neurological involvement is common, GI involvement more commonly results in a fatal outcome (often perforation after necrosis). Neurologic involvement can also occur as headache, hemiparesis, aphasia, and cranial nerve involvement, but is less likely to cause fatality.
20- This patient likely has which of the following complaints in addition to the photo shown?
A. Proximal muscle weakness
B. Immunodeficiency
C. Numerous telangiectasias
D. Hematuria
E. Dysphagia
Correct choice: A. Proximal muscle weakness
Explanation:. Ragged cuticles (Samitz sign) is classically seen in dermatomyositis, but can also be seen in other autoimmune connective tissue diseases.
The other features listed aren’t as commonly seen in dermatomyositis. Hematuria can be seen in HSP which has palpable purpuric papules and macules on extensor surfaces and buttocks. Dysphagia can be seen in systemic sclerosis & Plummer-Vinson syndrome. Numerous telangiectasias can occur in Hereditary Hemorrhagic Telangiectasia or Ataxia-Telangiectasia. Immunodeficiency can occur in Wiskott-Aldrich syndrome, Chronic Granulomatous disease, and Severe combined immunodeficiency.
21- If a patient had a history of recurrent ischemic strokes and presents with livedo reticularis, which of the following labs abnormalities would you most likely find, assuming it is clinically relevant?
A. Platelets of >800,000/microliter
B. Low levels of protein S
C. Anti-beta-2 glycoprotein antibodies
D. Anti-heparin antibodies
E. Factor V mutation
Correct choice: C. Anti-beta-2 glycoprotein antibodies
Explanation: The correct answer is C, which is one of 3 labs in the antiphospholipid antibody syndrome. The combination of livedo reticularis (or livedo racemosa) with neurological disease, especially ischemic strokes, points to Sneddon Syndrome, which may show a positive workup for antiphospholipid antibody syndrome.
22- A patient presents to clinic with a new onset of bullous lesions on his ankles. He notes immediately prior to the onset of these lesions, he was taking a walk through a local park. Which of the following is the most common association?
A. IgA gammopathy
B. Chronic Lymphocytic leukemia
C. History of scabies infestation
D. Thrombocytopenia
E. Exfoliative Toxin A
Correct choice: B. Chronic Lymphocytic leukemia
Explanation: This question stem is describing a bullous arthropod reaction. This is typically seen in the setting of CLL. IgA gammopathy could result in IgA pemphigus but the sudden nature of these lesions would suggest against this. Exfoliative Toxin A would be associated with bullous impetigo but the time course of the lesions starting immediately after a walk in the park would suggest against this dx. Acropustulosis of infancy has been associated with prior scabies infection. Thrombocytopenia has not been directly associated with bullous lesions.
23- A 65-year old male has long standing diabetes mellitus. Which of the following cutaneous lesions are most likely to develop in this patient?
A. Rapidly developing tense blisters on the hands and feet
B. Sudden crops of firm, yellowish papules with a rim of erythema
C. Bilateral red-brown atrophic macules on shins
D. Waxy skin over the dorsal hands and feet
E. Firm, annular erythematous plaques with central clearing
Correct choice: C. Bilateral red-brown atrophic macules on shins
Explanation: Diabetic Dermopathy, or “shin spots”, are the most common manifestation of diabetes mellitus. It presents as brown atrophic macules and patches on the shins. These occur in approximately 50% of diabetics and are due to microangiography. These patients are at risk for other vasculopathy-associated manifestations such as nephropathy, neuropathy, and retinopathy. Unfortunately, there are no effective treatments for the condition. The other answer choices represent less common cutaneous manifestations of diabetes, including granuloma annulare (firm, annular erythematous plaques with central clearing), eruptive xanthomas (sudden crops of firm, yellowish papules with a rim of erythema), bullosis diabeticorum (rapidly developing tense blisters on the hands and feet), and scleroderma-like skin (waxy skin over the dorsal hands and feet).
24- An 80 year old woman presents to your office with new-onset, yellow, thin, smooth plaques around her eyes, body folds, and trunk. Which of the following underlying conditions is associated with this presentation?
A. Acute myelogenous leukemia (AML)
B. Multiple myeloma
C. Gastric adenocarcinoma
D. Castleman’s disease
E. Thymoma
Correct choice: B. Multiple myeloma
Explanation: Diffuse normolipemic plane xanthoma (DNPX) was first described by Altman and Winkelmann in 1962. It is a rare and non-inherited form of xanthomatosis. Clinically, the dermatosis is characterized by the presence of symmetric yellowish-orange plaques that favor the neck, upper trunk, flexural folds and periorbital region. It has been recognized to be associated with hematological diseases, especially with multiple myeloma and monoclonal gammopathy. Thymoma and Castleman’s disease are associated with paraneoplastic pemphigus. Gastric adenocarcinoma is associated with acanthosis nigricans and tripe palms, as well as the suddent onset of multiple seborrheic keratoses (Sign of Leser-Trelat). AML is associated with Sweet’s syndrome.
25- A patient is referred for these findings. Which study is the most appropriate in this setting?
A. Ceruloplasmin
B. Arsenic level
C. Iron
D. Chest X-ray
E. BUN/Cr
Correct choice: A. Ceruloplasmin
Explanation: This kodachrome is of blue lunulae, which can occur with zidovudine, silver, or Wilson’s disease. The test for Wilson’s disease would be decreased ceruloplasmin. It is caused by an autosomal recessive ATP7B gene defect (copper-transporting ATPase). It causes an accumulation of copper, resulting in blue lunulae, Kayser-Fleishcher rings, neurologic sequelae, and liver failure. BUN/Cr would evaluate for CKD in half and half nails. Chest X-ray could be used for evaluating pulmonary disease in clubbing. Iron deficiency anemia can cause koilonychia. Arsenic would cause Mee’s lines which would present with transverse white lines.
26- A 22 y/o patient presents with the following dermatologic finding. A biopsy is performed with the below findings. Which of the following is the appropriate first step?
A. Check ASO titer
B. Check platelet count
C. Check HgbA1C
D. Excision of lesion
E. Check TSH
Correct choice: A. Check ASO titer
Explanation: This kodachrome and histologic photos show erythema nodosum. Clinically, it presents with erythematous, tender nonulcerating nodules that progress to a violaceous bruise-like color. Histologically it presents with a septal panniculities; one would see septal edema, a mixed inflammatory infiltrate, and widened septa with fibrosis.
Streptococcus is the most common infectious cause of erythema nodosum (EN) and therefore ASO titers should be evaluated as a first line diagnostic test for EN. An evaluation of other infectious etiologies, a medication review, and a thorough personal and family history for rheumatologic illnesses should be taken. Excision of erythema nodosum would be an inappropriate first choice, as spontaneous resolution of EN typically occurs within 3-6 weeks. If bruising is suspected, platelet count may be evaluated for thrombocytopenia. However, the pathologic image here indicates a septal panniculitis, which would not occur in thrombocytopenia. HgbA1c could be important if necrobiosis lipoidica diabeticorum is suspected. This would present with well demarcated, firm, waxy, yellowish brown plaques, which is not seen in this photo. TSH would not be the first step in the work-up for erythema nodosum.
27- A patient presents with thinning of the lateral third of her eyebrows. Which of the following is not associated with this finding?
A. Hyperthyroidism
B. Mycosis fungoides
C. Syphillis
D. Trichotillomania
E. Leprosy
Correct choice: A. Hyperthyroidism
Explanation:The sign of Hertoghe or Queen Anne’s sign is a thinning or loss of the outer third of the eyebrows. It is typically seen in HYPOthyroidism not HYPERthyroidism. Hertoghe’s sign can be
seen in Ulerythema ophroygenes, hypothyroidism, leprosy, alopecia mucinosa, folliculotrophic MF, ectodermal dysplasia, trichotillomania, and syphilis.
28- Hyperkeratotic follicular nasal papules have been described as a paraneoplastic sign in the setting of which neoplasm?
A. Multiple myeloma
B. Castleman’s tumor
C. AML
D. Adenocarcinoma of the lung
E. Renal cell carcinoma
►A
Hyperkeratotic follicular nasal papules have been described as a paraneoplastic phenomenon in the setting of multiple myeloma. AML is associated with Sweet’s syndrome and Castleman’s tumor is associated with paraneoplastic pemphigus.
29 - Which of the following may be associated with Graves‟ disease?
A. Dermatitis herpetiformis
B. Geographic tongue
C. Hypohidrosis
D. Madarosis
E. Thick, pale lips
►A
Graves‟ disease is a thyrotoxic condition that results from the production of thyroid-stimulating immunoglobulins (TSI) by stimulated B lymphocytes. The TSI bind to the thyroid-stimulating hormone (TSH) receptor and mimic TSH thereby stimulating thyroid growth and thyroid hormone overproduction. Signs and symptoms of Graves‟ disease include goiter, tachycardia, exophthalmos, tremor, sweating, palpitations, smooth moist skin, diarrhea, sleeplessness, irritability, and weight loss. Autoimmune cutaneous disease may also be associated with Graves‟
disease including vitiligo, dermatitis herpetiformis, herpes gestationis, and pemphigus vulgaris. Cutaneous manifestations of hypothyroidism include xerosis, hyperhidrosis, yellowish hue, myxedema, and purpura. The hair may be dry, brittle and coarse; alopecia may be diffuse and/or involve the lateral eyebrow (madarosis).
30 -A patient with gluten-sensitive enteropathy presents with vesicles on the extensor surfaces of the extremities. What findings are most likely on a perilesional biopsy?
A. Granular IgA at the dermoepidermal junction on direct immunofluorescence
B. Linear C3 and IgG at the dermoepidermal junction on direct immunofluorescence
C. Linear IgA surrounding vessels on direct immunofluorescence
D. Pautrier”s micro-abscesses in the epidermis on H & E
E. Leukocytoclastic vasculitis
►A
This patient has dermatitis herpetiformis (DH). DH is characterized by itchy papulovesicles on extensor surfaces of the extremities. Neutrophilic infiltrates at the dermal papillae with vesicle formation are found on histopathologic examination. On immunofluorescence, granular IgA (directed against transglutaminase) deposits are found in perilesional skin. Over 90% of patients have gluten-sensitive enteropathy of varying severity. Dapsone is almost universally therapeutic for the skin disease, but not the enteropathy. A gluten free diet, although difficult to m aintain, treats the enteropathy. The majority of DH patients have the HLA class II DQ2 genotype. On indirect immunofluorescence, one may find antigliadin, antiendomyseal or antireticulin antibodies. Autoimmune thyroid disease (especially Hashimoto”s thyroiditis), enteropathyassociated T-cell non-Hodgkin”s lymphoma and insulin-dependent diabetes are the most common autoimmune associations. Pautrier”s micro-abscesses are found in mycosis fungoides. Leukocytoclastic vasculitis is not a feature of DH.
31- A 64-year-old woman presents with lichenified keratotic plaques on the bilateral lower extremities. Biopsy reveals suppurative inflammation and collagen extending from the dermis through the epidermis. Which is the most likely associated systemic disease:
A. Congestive Heart Failure
B. Hepatitis C Infection
C. Hypothyroidism
D. Chronic kidney disease
E. Castleman‟s disease
►D
Chronic kidney disease is associated with perforating disorders. These disorder are characterized by pruritus and a biopsy revealing collagen and inflammatory cells broaching the epidermis. The other disorders are not closely associated with perforating diseases.
32- A patient presents with mild mental retardation, infertility, joint contractures, short stature, ichthyosis, and sparse hair with trichoschisis. All of the following are true regarding this patient’s condition EXCEPT:
A. This syndrome is inherited in an autosomal recessive manner
B. If photosensitivity is a feature, gonad size is likely normal
C. Patients may have associated cataracts
D. The syndrome is caused by impaired nucleotide excision repair
E. Perifoveal glistening white dots are a feature
►E
The patient described has IBIDS syndrome (Ichthyosis, Brittle hair, Intellectual impairment, Decreased fertility, and Short stature), or Tay’s syndrome. This syndrome is autosomal recessive, caused by mutations in the ERCC2/XPD or ERCC3/XPB genes, resulting in impaired nucleotide excision repair. Cataracts may be a feature. If photosensitivity is a feature (PIBIDS), gonal size is likely normal. Perifoveal glistening white dots are a feature of Sjorgen-Larsson Syndrome, not IBIDS.
33- A patient with congenital hypertrophy of retinal epithelium is most likely to have:
A. An autosomal dominant mutation in the MSH2 gene
B. Pheochromocytoma
C. Adenomatous polyposis
D. Tram-track calcifications on head radiograph
E. Peg-shaped teeth
►C
Congenital hyperpigmentation of the retinal pigment (CHRPE) is an early feature of Gardner syndrome (GS). It is found in approximately 60% of patients with GS. GS is an autosomal dominant disorder characterized by precancerous intestinal polyposis and subsequent adenocarcinoma of the gastrointestinal tract. Cutaneous manifestations include epidermoid cysts, osteomas, desmoids and fibrous tumors. A mutation in the adenomatous polyposis coli (APC) gene, a tumor suppressor gene, is responsible for the disease. Most patients develop colon carcinoma by the 2nd or 3rd decade. Therefore, prophylactic colectomy is warranted. Mutations in the MSH2 gene are found in Muir-Torre syndrome. Pheochromocytomas are found in multiple endocrine neoplasia (MEN) syndromes IIa and IIb. Tram track calcifications are found in SturgeWeber syndrome. Peg-shaped teeth are found in multiple syndromes including ectodermal dysplasia.
34- A 14-year-old female presents with gastrointestinal pain and seizures. Laboratory studies reveal hyponatremia. Which of the following medications was most likely to induce her systemic symptoms:
A. Phenytoin
B. Diphenhydramine
C. Griseofulvin
D. Morphine
E. Acetaminophen
►C
Acute intermittent porphyria (AIP) is associated with colicky abdominal pain, neurologic symptoms, and SIADH. This disorder is caused by deficiency of porphobilinogen deaminase. AIP may be exacerbated by drugs including barbiturates, sulfonamides, and griseofulvin, among others.
35- Which of the following is not a feature of Cronkhite-Canada syndrome?
A. Diarrhea
B. Alopecia
C. Lung carcinoma
D. Dystrophic nails
E. Hyperpigmented macules
►C
Cronkhite-Canada syndrome is a rare, non-familial disease characterized by patchy alopecia, nail changes or loss, lentigines, inflammatory polyps, abdominal pain, and a protein losing enteropathy.
- -Which of the following is a paraneoplastic disease most often associated with lung carcinoma?
A. Hypertrichosis lanuginosa acquisita
B. Dermatomyositis
C. Acanthosis nigricans
D. Paraneoplastic pemphigus
E. Erythroderma
►A
Hypertrichosis lanuginosa acquisita is the abrupt onset of downy, soft, non-pigmented hair of the face, trunk, and extremities. It may have an associated glossitis. It is associated with underlying lung carcinoma and may resolve with treatment of the underlying malignancy.
- Which of the following is true regarding piebaldism?
A. It is caused by a mutation in the GJB2 gene
B. It is caused by defective metabolism of phytanic acid
C. It is caused by a defect in a protein subunit of a kinase that activates NFkappaB
D. It is caused by a deficiency of fatty aldehyde dehydrogenase
E. It is caused by a mutation in the proto-oncogene c-KIT
►E
Piebaldism is caused by an autosomal dominant mutation of the proto-oncogene c-KIT which encodes tyrosine a tyrosine kinase receptor on melanocytes, preventing activation by steel factor.
- A- middle-aged gentleman who avoided healthcare dies suddenl y from gastrointestinal hemorrhage. Post-mortem examination reveals multiple soft blue compressible tumors on the trunk, arms, and tongue. Blue rubber bleb nevus syndrome is caused by a mutation in what gene?
A. VMCM1
B. ENG
C. VEGF
D. PTEN
E. RET
►A
Blue rubber bleb nevus syndrome is a rare sporadic or autosomal dominant disorder with soft compressible blue tumors on the trunk and arms. Nocturnal pain is characteristic. Gastrointestinal hemangiomas can cause hemorrhage. Mutations in the VMCM1=TEK=Tie -2 gene are reported. It is also knkow n as VMCM syndrome.
39- Which of the following statements regarding porphyrias is TRUE?
A. Elevated uroporphyrins are found in the red blood cells of hepatoerythropoietic porphyria
B. Delta aminolevulenic acid is the only oxidized porphyrin
C. Acute intermittent porphyria is the most common form of porphyria
D. Griseofulvin is safe for those with variegate porphyria
E. Plasma fluoresces at 410 nm in patients with variegate porphyria
►B
Acute intermittent porphyria (AIP) is the second most common porphyria and is caused by a deficiency in porphobilinogen (PBG) deaminase, which is located in the cytosol. Patients suffer from colicky pain, paralysis and psychiatric disorders. There are no specific skin manifestations. PBG and aminolevulenic acid (ALA) are elevated in the urine. Attacks are precipitated by medications such as barbiturates, estrogen, griseofulvin, and sulfonamides as well as starvation, fever and infection. Treatment includes glucose loading and hematin infusion.
Congenital erythropoietic porphyria (CEP) or Günter‟s disease is caused by a defect in uroporphyrinogen III synthase, which is found in the cytosol. Patients are extremely photosensitive and erythema, blistering and scarring result. Patients present with red urine early in life along with hypertrichosis and red-stained teeth that fluoresce. Uroporphyrins (URO) are much high than coproporphyrins (COPRO) in the urine. URO is found in the red blood cells (rbcs) and COPRO is found in the stool. The rbcs display stable fluorescence.
Porphyria cutanea tarda (PCT) is the most common porphyria and is caused by a deficiency (usually sporadic) in uroporphyrinogen decarboxylase, which is found in the cytosol. Patients present with photosensitivity and blistering of sun-exposed areas, especially the dorsal hands. Hypertrichosis and sclerodermoid changes may occur as well. Liver disease (hepatitis C or alcoholic cirrhosis) is often present and hemochromatosis may be associated. Urine may fluoresce pink or coral-red with Wood‟s lamp. URO>COPRO in the urine and low levels of COPRO are found in the stool. Treatments include phlebotomy, antimalarials, and therapy for liver disease if appropriate.
Hereditary coproporphyria (HCP) is caused by a deficiency in coproporphyrinogen oxidase, which is found in the mitochondria. One-third of patients are photosensitive, and patients suffer gastrointestinal and neurological symptoms similar to AIP. Urine COPRO is elevated only with attacks, and COPRO is present in the stool.
Variegate porphyria is the result of decreased activity of protoporphyrinogen oxidase, which is present in the mitochondria. It combines the skin lesions of PCT with the systemic manifestations of AIP. Urine COPRO:URO is 1:1 or COPRO> URO to distinguish it from PCT, and PROTO is found in the stool. The plasma fluoresces at 626nm. Precipitators and treatments are similar to AIP.
Erythropoietic protoporphyria (EPP) is caused by ferrochetalase deficiency, which is present in the mitochondria. Patients experience immediate burning of the skin with sun exposure. Protoporphyrin IX, the only oxidized porphyrin in the heme pathway and absorbs in the Soret band (400-410nm). Patients have erythematous plaques in a photo-distribution. Urine porphyrins are normal. PROTO is
found in the rbcs and the stool. Excessive porphyrins deposited in the liver lead to gallstones and cirrhosis. Beta carotene may helpful.
Hepatoerythropoietic porphyria (HEP) is essentially a homozygous form of PCT, with deficiency in uroporphyrinogen decarboxylase. It is clinically similar to CEP with red urine and hypertrichosis, vesicles and scarring of sun-exposed skin. URO is present in the urine and COPRO in the stool. PROTO is present in rbcs which distinguishes it from CEP, which was URO in rbcs.
40- The expected histology of a biopsy take from the lesion shown in the image would
A. Increased mucin
B. Schumann bodies
C. Granulomatous infiltrate localized to the papillary dermis
D. Elastic fiber degeneration
E. Collagen degeneration
►E
The histologic findings of necrobiosis lipodica diabeticorum are: dermal granulomatous inflammation in a horizontal pattern, collagen degeneration, and normal or atrophic epidermis.
41 -Squamous cell carcinoma is seen in which syndrome?
A. Gorlin syndrome
B. Rombo syndrome
C. Nicolau-Balus syndrome
D. Rasmussen syndrome
E. Bazex syndrome (Acrokeratosis paraneoplastica)
►E
Basex syndrome, also Acrokeratosis paraneoplastica, presents with symmetric erythematous, nearly violaceous, psoriasiform dermatoses of the hands, feet, ears and nose. The syndrome is nearly associated with an underlying malignancy, usually squamous cell carcinoma of the upper aerodigestive tract.
42- All of the following statements are true regarding this condition EXCEPT:
A. Diabetes or glucose intolerance is found in 20% of these patients
B. This condition may be associated with cutaneous anesthesia, hypohidrosis, and partial alopecia
C. There is no impact of tight glucose control on the likelihood of developing this condition
D. 0.3-3% of diabetics have this skin condition
E. This condition is associated with increased dermal mucin
►E
Necrobiosis Lipoidica Diabeticorum (NLD) is found in 0.3-3% of diabetics. Approximately 20% of NLD patients have diabetes or glucose intolerance. It presents with single or mul tiple redbrown papules which progress to sharply demarcated yellow-brown atrophic, telangiectatic plaques with violaceous, irregular borders; common sites include the shins. Cutaneous anesthesia, hypohidrosis, and partial alopecia can be found. Pathology shows palisading granulomas containing degenerating collagen (necrobiosis); with NO increase in dermal mucin. There is no impact of tight glucose control on the likelihood of developing NLD.
43- Which of the following is NOT associated with Hepatitis C disease?
A. Mixed cryoglobulinemia
B. PCT
C. Single-stranded RNA viridae
D. Leukocytoclastic vasculitis
E. Single-stranded DNA viridae
►E
Hepatitis C virus (HCV) is a single-stranded RNA virus that is a member of the flaviviridae family. Approximately 20-30% of patients develop symptoms with acute infection and 70% will progress to chronic disease. Porphyria cutanea tarda or PCT is associated with HCV in a substantial percentage of patients; in one study, antibodies to HCV were found in 82% of PCT patients. Up to 80% of mixed cryoglobulinemia (MC) cases are associated with HCV. The incidence of lichen planus in HCV patients varies from region to region (0.1-35%). The histopathology of MC lesions is leukocytoclastic vasculitis. Another relatively common association is polyarteritis nodosa (PAN) which is also related to hepatitis B infection. Finally, generalized pruritis is a common complaint of HCV patients.
44- All of the following are seen more commonly in Crohn’s disease than in ulcerative colitis EXCEPT:
A. Oral cobblestoning
B. Polyarteritis nodosa
C. Pyostomatitis vegetans
D. Perineal fistulas
E. Perineal fissures
►C
Pyoderma vegetans consists of vegetating plaques and vesicopustules of intertriginous areas than heal with hyperpigmentation. When the process involves mucosal surfaces it is called pyostomatitis vegetans. These processes are both associated with ulcerative colitis, not Crohn’s disease.
45- Patients with plexiform neuroma and NF I who also have JXG are at increased risk for developing:
A. Juvenile chronic myelogenous leukemia
B. Non-Hodgkin’s Lymphoma
C. Esophageal cancer
D. Breast cancer
E. Medullary carcinoma of the thyroid gland
►A
Patient with NF 1 who have multiple juvenile xanthogranulomas are at 20 times greater risk for developing juvenile chronic myelogenous leukemia.
-46. The peak sensitivity to prophyrins occurs at which wavelengths?
A. 220-290nm
B. 290-320nm
C. 320-400nm
D. 400-410nm
E. 410-450nm
►D
The Soret band (400-410 nm) is the portion of ultraviolet wavelengths at which most porphyrins are most sensitive.
- A patient presents with plane/palmar xanthomas. The most likely genetic disorder would be:
A. familial lipoprotein lipase deficiency
B. Familial hypertriglyceridemia
C. Familial hypercholesterolemia
D. Familial dysbetalipoproteinemia
E. Cerebrotendinous xantomatosis
►D
Patients with Type III hyperlipidemia have both elevated triglyceride levels and cholesterol levels in the plasma. A genetic basis for the primary disorder, familial dysbetalipoproteinemia, has been well established. These patients present as adults with premature atherosclerosis and xanthomas, particularly plane (palmar) xanthomas.
48 -A 70-year old female develops erythema with fine adherent scale on acral skin that progresses to keratoderma and eventually a more generalized psoriasiform dermatitis. What is the most likely underlying malignancy?
A. Squamous cell carcinoma of the larynx
B. Adenocarcinoma of the colon
C. Adenocarcinoma of the breast
D. Squamous cell carcinoma of the vagina
E. Medullary thyroid carcinoma
►A
Acrokeratosis Paraneoplastica (aka Bazex) is always associated with malignancy. Most commonly, the associated malignancy is a squamous cell carcinoma of the upper aerodigestive tract. The skin disease usually follows the course of the malignancy.
49- Patients with Werner‟s syndrome typically experience which of the following types of cardiac disease?
A. Hypertrophic cardiomyopathy
B. Aortic aneurysms
C. Premature atherosclerosis
D. Cardiomegaly
E. Mitral valve prolapse
►C
Werner‟s syndrome or Adult Progeria is caused by autosomal recessive mutations in WRN (Recql2) gene, which encodes DNA helicase. This defect leads to defects in DNA repair and replication. Patients prematurely age and essentially experience many diseases of aging early in childhood and teen years. They exhibit tight atrophic skin, relatively large heads for body size, leg ulcers and cannities. Patients experience early, accelerated atherosclerosis leading to death by myocardial infarction. In addition, type II diabetes, cataracts, osteoarthritis, osteoporosis and hypogonadism are features.
50- A patient with end stage renal disease undergoes an MRI examination with contrast. He subsequently develops woody indurated plaques on the extremities. Fatalities seen in nephrogenic systemic fibrosis are due to what underlying process?
A. Fibrosis of respiratory muscles
B. Fibrosis of cardiac smooth muscle
C. Fibrosis of the gastrointestinal tract
D. Fibrosis of cerebral arteries
E. Fibrosis of the liver
►A
Nephrogenic systemic fibrosis is a chronic fibrosing disorder seen most commonly in the setting of renal failure. There is a reported association also with gadolinium as contrast in an MRI. Patients present with woody indurated plaques or nodules primarily of the extremities, with occasional involvement of the trunk. Fulminant and fatal disease is rare, occurring in about 5% of cases. Death is due to impaired ventilation secondary to fibrosis of respiratory muscles.
51- Which of the following skin findings is most closely linked to hepatocellular carcinoma as a paraneoplastic syndrome?
A. Pityriasis lichenoides
B. Pityriasis alba
C. Pityriasis amiantacea
D. Pityriasis rotunda
E. Pityriasis rosea
►D
Pityriasis rotunda is an dermatosis that features characteristic discrete, circular, scaly, brown patches on the trunk and extremities. Pityriasis rotunda may be associated with systemic diseases in certain racially predisposed groups (blacks), and has been linked to hepatocellular carcinoma.
52 -A 20-year-old male develops an eruption of 100’s of red-brown yellowish papules with involvement of the mucous membranes. He has no lymphadenopathy. The most likely diagnosis is:
A. Montgomery’s syndrome
B. Benign cephalic histiocytosis
C. Rosai-Dorfman disease
D. Necrobiotic xanthogranuloma
E. Multicentric reticulohistiocytosis
►A
This patient has Montgomery’s syndrome, or xanthoma disseminatum, which is characterized as a benign normolipidemic but disfiguring condition that demonstrates an eruption of 100’s of redbrown yellowish papules and plaques that may involve the mucous membranes. These patients should be evaluated for diabetes insipidus. Benign cephalic histiocytosis shows brownish-yellow papules on the upper face. Rosai-Dorfman, or sinus histiocytosis with massive lymphadenopathy, features polymorphic papules and plaques in the first two decades with fever, increased ESR, and cervical lymphadenopathy. Necrobiotic xanthogranuloma is characterized by red-orange plaques that may ulcerate. Finally, multicentric reticulohistiocytosis features coral beading around the fingers and is associated with arthritis mutilans and malignancy.
53- Which of the following porphyria cutanea tarda associations has a direct relationship to the level of urine uroporphyrins?
A. Dystrophic calcifications
B. Estrogen levels
C. Sclerodermoid changes
D. RBC fluorescence
E. Hypertrichosis
►C
Connective tissue dystrophic calcifications, increased estrogen levels, hypertrichosis and sclerodermoid changes are all associated with porphyria cutanea tarda, but sclerodermoid changes are the only finding with a direct relationship to urine uroporphyrin levels. Urine fluorescence occurs in PCT, not RBC fluorescence.
54- A 55 year old patient presents with new onset brown macules on arms, legs, face and palms. She gives a 3 month history of diarrhea, abdominal cramps, weight loss and protein-losing enteropathy. The most likely diagnosis is:
A. Peutz-Jeghers syndrome
B. Cowden disease
C. Ulcerative colitis
D. Cronkhite-Canada syndrome
E. Plummer-Vinson syndrome
►D
Cronkhite-Canada is an aquired disease characterized by the development of polyps throughout the GI tract. Patients can present with hyperpigmented macules as well as the sequella of GI malabsorption. The malignant transformation of polyps can occur.
55- Which of the following diseases is caused by an enzymatic defect that occurs in the mitochondria?
A. Porphyria Cutanea Tarda
B. Acute Intermittent Porphyria
C. Congenital Erythropoeitic Porphyria
D. Erythropoeitic Protoporphyria
E. Hepatoerythropoetic Porphyria
►D
Defects in many of the enzymes involved in heme synthesis are responsible for porphyrias. The first and last three steps of the heme synthesis pathway occur in the mitochondria.
56 -Which one of the following clinical findings suggests the diagnosis of multiple myeloma?
A. Sudden eruption of seborrheic keratoses
B. Hyperkeratotic follicular papules on the nose
C. Erythema, vesicles, and erosions in periorifical and acral areas
D. Concentric erythematous rings with trailing scale on trunk and extremities
E. Tripe palms
►B
Hyperkeratotic follicular nasal papules are associated with multiple myeloma. A sudden increase in number and size of seborrheic keratoses is called the sign of Lesser-Trelat and can be seen in gastric or colon carcinoma. Erythema and vesiculation in periorificial and acral areas is characteristic of necrolytic migratory erythema most often associated with pancreatic cancer.
Concentric erythematous rings with trailing scale is a feature of erythema gyratum repens most commonly seen in lung cancer. Tripe palms with AN more likely gastric whereas without AN more likely lung.
-All of the following disorders have an increased risk of systemic malignancy except
A. Rothmund Thompson syndrome
B. Cockayne syndrome
C. Bloom syndrome
D. Werner syndrome
E. Xeroderma pigmentosum
►B
Patients with Rothmund thompson have early photosensitivity and poikilodermatous skin changes, juvenile cataracts, skeletal dysplasias, and a predisposition to osteosarcoma and skin cancer. Patients with Bloom syndrome have an increased risk for G I malignancies and lymphoma. Patients with xeroderma pigmentosum can develop ocular melanoma. Patients with Werner syndrome can develop thyroid and hematologic malignancies, sarcomas,and meningiomas. Cockayne syndrome patients have photosensitivity, short stature, premature aging, visual problems, neurologic deficits, but no internal malignancies.
58- A 6 month-old has a verrucous plaque on the mucosal surface of the lower lip. Skin biopsy is consistent with Riga-Fede disease. You should refer the patient to:
A. An ophthalmologist
B. A gastroenterologist
C. A neurologist
D. A hematologist
E. An otolarngologist
►C
Riga-Fede disease is a benign ulcerative granulomatous process that occurs in reaction to chronic, repetitive trauma of the oral mucosa by the teeth. Clinically, it appears as firm, verrucous plaques. It may be associated with an underlying developmental anomaly or underlying neurologic disorder.
59 -A 64-year old woman presents with scalloped erosions in the dermatome of the first branch of the trigeminal nerve. Biopsy would reveal which of the following histologic findings:
A. Asteroid Bodies
B. Caterpillar Bodies
C. Cowdry Type A Bodies
D. Donovan Bodies
E. Dutcher Bodies
►C
Varicella zoster virus is the causative agent in chicken pox and shingles, where recrudescence from latency in the dorsal root ganglion causes a dermatomal distribution of painful vesicles and erosions. Biopsy of herpes viruses reveals cells with multinucleation, margination of the chromatin, and Cowdry Type A bodies, which are intranuclear eosinophilic amorphous bodies surrounded by a clear halo.
60 -The most common autoimmune disease associated with hepatitis C is:
A. Autoimmune thyroiditis
B. Myasthenia gravis
C. Aplastic anemia
D. Lymphocytic sialadenitis
E. Rheumatoid arthritis
►A
The most common autoimmune disease seen in association with Hepatitis C is autoimmune thyroiditis.
61-All of the following are true regarding Cockayne Syndrome EXCEPT:
A. Inheritance is autosomal recessive
B. It is caused by an inability to repair cyclobutane dimers
C. It is associated with basal ganglia calcifications
D. It is associated with retinal pigment degeneration with a “salt and pepper” appearance
E. None of these answers are incorrect
►E
Cockayne Syndrome is an autosomal recessive disorder caused by a mutation of an unknown gene that results in the inability to repair cyclobutane dimers induced by UV exposure. Cutaneous findings include photosensitivity, “bird-headed” facies, and “Mickey Mouse” ears. Statements A-D are true regarding the syndrome.
62 -With regards to patients with diabetes mellitus and skin, which of the following is most accurate?
A. Diabetic dermopathy is most commonly expressed on the forearms and feet
B. Scleredema most often presents on the tibial surfaces
C. Scleredema is the most common skin manifestation of diabetes mellitus
D. Less than 5% of patients with diabetes mellitus have necrobiosis lipoidica
E. Less than 5% of patients with necrobiosis lipoidica have diabetes mellitus
►D
Patients with diabetes mellitus (DM) may develop thickened skin and tightened joints. Scleredema is occasionally seen in type II diabetics and presents with a peau d‟orange texture and appearance on the upper back. Perhaps as many as 20% of those with necrobiosis lipoidica diabeticorum(NLD) have diabetes, but only 0.3% to 3% of diabetics have NLD. The most common skin manifestation of DM is diabetic dermopathy, or skin hyperpigmentation, normally on tibial surfaces. Eruptive xanthomas, which are associated with high triglycerides are occasionally seen as are generalized or perforating granuloma annulare.
79- The patient demonstrated in figure would have:
A. LDL receptor deficiency
B. Decreased chylomicrons
C. Elevated triglycerides
D. Decreased LDL
E. Decreased triglycerides
►C
This patient has eruptive xanthomas, a condition which is classically associated with elevated serum triglycerides.
104- All of the following are true regarding the condition pictured EXCEPT:
A. Perilesional direct immunofluroescence shows granular IgA in the dermal papillae and at the dermoepidermal junction
B. Only 20% of patients have a gluten-sensitive enteropathy
C. It is associated with HLA-DQ2, HLA-DR3, and HLA-B8
D. It is associated with Hashimoto’s thyroiditis
E. Cutaneous findings are due to autoantibodies to epidermal transglutaminase
►B
All of the statements regarding dermatitis herpetiforms, or “Duhring’s Disease,” are true except for statement B. Virtually all DH patients have gluten-sensitive enteropathy, although only 20% of them have symptoms (such as diarrhea, steatorrhea, weight loss, bloating, and malabsorption).
112- The clinical findings in the figure are associated with which disorder?
A. Grave”s disease
B. Myasthenia gravis
C. Dermatomyositis
D. Sarcoidosis
E. Rheumatoid Arthritis
►A
This slide shows pretibial myxedema in a patient with Grave‟s disease.
Pretibial myxedema is a form of diffuse mucinosis in which there is an accumulation of excess glycosaminoglycans in the dermis and subcutis of the skin. Glycosaminoglycans, also called mucopolysaccharides, are complex carbohydrates that are important for tissue hydration and lubrication. The main glycosaminoglycan in pretibial myxedema is hyaluronic acid, which is made by cells called the fibroblasts. Pretibial myxedema is also known as localized myxedema, thyroid dermopathy, and infiltrative dermopathy. It is most commonly seen on the shins (pretibial areas) and is characterized by swelling and lumpiness of the lower legs.
135- What is the most common malignancy associated with this condition at this location?
A. Breast cancer
B. Gastric cancer
C. Thyroid cancer
D. Pancreatic cancer
E. Melanoma
►B
Malignant acanthosis nigricans usually presents with sudden onset and is rapidly progressive. It may be associated with diffuse keratodermas of the palms and soles or eruptive seborrheic keratoses.
145 - A complication seen in women of childbearing age with the condition in the figure is:
A. Atrial septal defect
B. Pulmonary lymphangioleiomyoma
C. Gastrointestinal bleeding
D. Lisch nodules
E. Pulmonary emboli
►B
This picture demonstrates a shagreen patch in a patient with Tuberous sclerosis. A complication occurring in female patients of child-bearing age is pulmonary lymphangioleiomyoma.
150- The organism that causes this infection shown in the figure is:
A. T. tonsuran
B. C. immitis
C. C. albicans
D. P. aeruginoas
E. T. verrucosum
►C
The organism that causes erosio interdigitalis blastomycetica is candida albicans.
