Vesicular and Bullous Dermatosis MCQs

A 29 year old female patient is diagnosed with fogo selvagem. The likely possibility that this person with die within two years is : 1

90%

2

70%

3

40%

4

20%

5

10%

Q/Q(M)-482895 Report a Problem A 29 year old female patient is diagnosed with fogo selvagem. The likely possibility that this person with die within two years is : 3

40%

Patients with fogo selvagem have a 40% of dying within 2 years with they do not receive t reatment. These patient have burning lesions that is denuded with roll of pushed back upper epidermis at  periphery. Patients can also develop erythroderma Q/Q(M)-482895 Report a Problem The rate limiting step in the porphyria pathway is mediated by the enzyme: 1

Ferrochetalase

2

ALA dehydratase

3

ALA synthase

4

Uroporphyrinogen Uroporphyrinogen decarboxylase

5

Porphobilinogen deaminase

Q/Q(M)-474613 Report a Problem The rate limiting step in the porphyria pathway is mediated by the enzyme: 3

ALA synthase

ALA synthase mediates the first and rate-limiting rate -limiting step in the heme synthesis s ynthesis pathway. This step occurs in the mitochondria and allows for the conversion of Succinyl coA + glycine to delta aminolevilinic acid. Q/Q(M)-474613 Report a Problem The most common cause of contact dermatitis in the United States is: 1

Hair dyes

2

Nickel

3

Nail lacquers

4

Toxicodendrons

5

Rubber Q/Q(M)-478699 Report a Problem

The most common cause of contact dermatitis in the United States is: 1

4

Toxicodendrons

The most common cause of contact dermatitis in the t he USA are (in order of most to t o least common): toxicodendrons (poison ivy, oak, or sumac), nickel, fragrance, thimerosal, quarternium-15, neomycin, formaldehyde and the formaldehyde-releasing preservatives, bacitracin, and rubber compounds. Q/Q(M)-478699 Report a Problem Which of the following allergens is a non-formadelhyde releasing preservative with a low sensitization  potential? 1

Methylisothiazolone

2

Paraben

3

Bronopol

4

Quternium-15

5

DMDM hydantoin Q/Q(M)-482266 Report a Problem

Which of the following allergens is a non-formadelhyde releasing preservative with a low sensitization  potential? 2

Paraben

Paraben mix is a non-mercurial, non-formaldehydge releasing preservative with l ow sensitization  potential. Methylisothiazolone is a non-formaldehydge releasing preservative found in wash-on/washoff products. The others are formaldehyde-releasing preservatives. Q/Q(M)-482266 Report a Problem Which of the following is the most common photoallergen? 1

octyl dimethyl PABA

2

Dihydroxyacetone

3

Benzophenone

4

Oxybenzone

5

glyceryl thioglycolate Q/Q(M)-474140 Report a Problem

Which of the following is the most common photoallergen? 4

Oxybenzone

Oxybenzone is a UVA sunscreen and is the most common photoallergen. Octyle dimethyl PABA (Padimate O) and benzophenone are also sunscreen agents, and both can also cause contact dermatitis. Dihydroxyacetone is a sunless-tanning agent and glyceryl thioglycolate is found in permanent wave solutions. Q/Q(M)-474140 Report a Problem

2

The lamina densa is primarily composed of? 1

Connexins

2

Plectin

3

Collagen XVII

4

Collagen VII

5

Collagen IV Q/Q(M)-474603 Report a Problem

The lamina densa is primarily composed of? 5

Collagen IV

Collagen IV is located in the dermal matrix and forms the anchoring plaque. The anchoring plaque serves to anchor the lamina densa to the dermis via collagens 1,3 & 5. Q/Q(M)-474603 Report a Problem

Allergic contact dermatitis is characterized histologically by: 1

Psoriasiform dermatitis

2

Lichenoid infiltrate

3

Spongiosis

4

Parakeratosis

5

Granuloma Q/Q(M)-474601 Report a Problem

Allergic contact dermatitis is characterized histologically by: 3

Spongiosis

Spongiosis refers to the accumulation of edema fluid between keratinocytes, in some cases progressing to vesicle formation. The epidermis acquires a "spongy" appearance. This histopathologic pattern of inflammation is usually accompanied by an infiltrate of l ymphocytes within the epidermis and around superficial vessels. In addition to spongiosis, allergic contact dermatitis is typically accompanied by eosinophils. Q/Q(M)-474601 Report a Problem The primary autoantigen in pemphigoid gestationis is 1

Desmoplakin

2

BPAG1

3

BPAG2

4

Plakoglobin 3

5

Anchoring fibrils Q/Q(M)-476543 Report a Problem

The primary autoantigen in pemphigoid gestationis is 3

BPAG2

Herpes gestationsis is an autoimmune blistering disorder of pregnancy. It is characterized by urticarial  plaques and tense bullae. Antibodies to BPAG2 are thought to form as a result of an aberreant response to MHC antigens on the placenta. The maternal health is generally not affected, but infants are more commonly born premature. Remission is common following delivery, but it may recur with OCP's, menstruation, and subsequent pregnancies. Q/Q(M)-476543 Report a Problem Which type of porphyria disease is most likely to be associated with cholelithiasis? 1

Acute intermittent porphyria

2

Congenital erythropoeitic porphyria

3

Hereditary coproporphyria

4

Erythropoetic protoporphyria

5

Variegate porphyria Q/Q(M)-474592 Report a Problem

Which type of porphyria disease is most likely to be associated with cholelithiasis? 4

Erythropoetic protoporphyria

Erythropoeitic protoporphyria is caused by a defect in the enz yme ferrrochetalase. Patients present with immediate photosensitivity and subsequently develop waxy scarring on the face, hands and knuckles. In addition to abdominal pain, cholelithiasis and liver disease ma y also occur. The porphyria diseases associated with abdominal pain are acute intermittent i ntermittent porphyria, hereditary coproporphyria and variegate porphyria. Congenital erythropoeitc porphyria is associated with splenomegaly. Q/Q(M)-474592 Report a Problem The genetic predisposition for patients with pemphigus vulgaris is: 1

HLA-DRQ402

2

HLA-DR3

3

HLA-DR4

4

HLA-0505

5

HLA-Bw3 Q/Q(M)-482891 Report a Problem

The genetic predisposition for patients with pemphigus vulgaris is: 1

HLA-DRQ402

Patients with HLA-DRQ402 can be predisposed to the development of pemphigus vulgaris. Pemphigus vulgaris is a severe potentially fatal disease with blister formation on the skin and the mucosa 4

Q/Q(M)-482891 Report a Problem A patient who works in a photography developing lab complains of eczematous dermatitis on his hands. Which one of the following chemicals is he most likely allergic to? 1

Mercaptobenzothiazole

2

Lanolin

3

Epoxy resin

4

Parapheylenediamine

5

Colophony Q/Q(M)-482260 Report a Problem

A patient who works in a photography developing lab complains of eczematous dermatitis on his hands. Which one of the following chemicals is he most likely allergic to? 4

Parapheylenediamine

Photography solutions often contain parapheneylenediamine, or PPD. Mercaptobenzothiazole is a rubber accelerator found in shoes, rodenticides, and insecticides, and is the most common allergen of health care workers. Lanolin is a moisturizer moisturiz er found in cosmetics including aquaphor and eucerin. Epoxy resin is found in glues and marine cement and, if positive, has a high degree of clinical relevance. Colophony has the active ingredient of abietic acid and is found in pine rosin, violins, soaps, and adhesives. Q/Q(M)-482260 Report a Problem Characteristic eosinophilic abscesses are seen in: 1

Bullous drug

2

Pemphigus vegetans

3

Herpes gestationis

4

Incontinentia pigmenti

5

Paraneoplastic pemphigus

Q/Q(M)-474638 Report a Problem Characteristic eosinophilic abscesses are seen in: 2

Pemphigus vegetans

Eosinophilic abscesses with minimal to no spongiosis in a hyperplastic epidermis are characteristic of  pemphigus vegetans. Q/Q(M)-474638 Report a Problem

Antibodies in some forms of Stevens-Johnson bind to: 1

Desmoplakin I

2

Desmoglein 1

3

Desmoglein 3 5

4

Plakoglobin

5

Desmoplakin I and desmoglein 3 Q/Q(M)-474622 Report a Problem

Antibodies in some forms of Stevens-Johnson bind to: 1

Desmoplakin I

Antibodies against desmoplakin I and II have been found in Stevens-Johnson. Q/Q(M)-474622 Report a Problem This is found in both desmosomes and adherens junctions: 1

Alpha-catenin

2

Desmoglein 3

3

Desmoglein 1

4

Plakoglobin

5

Lamin Q/Q(M)-474658 Report a Problem

This is found in both desmosomes and adherens junctions: 4

Plakoglobin

Plakoglobin is a plaque protein of the armadillo family that is found in desmosomes; it can also substitute for beta-catenin in adherens junctions. Plakoglobin co-precipitates with desmogleins. It is 85 kDa. Q/Q(M)-474658 Report a Problem The dimethylglyoxime test is used to detect the presence of: 1

Gold

2

Silver

3

Mercury

4

Nickel

5

Latex Q/Q(M)-474612 Report a Problem

The dimethylglyoxime test is used to detect the presence of: 4

Nickel

1% dimethylglyoxime is added to to the metal. If a red precipitate forms, there is >1:10000 nickel content. Q/Q(M)-474612 Report a Problem Which pair is incorrect? 1

Desmoglein 1 : 160 kDa 6

2

Desmoplakin I : 250 kDa

3

Type VII collagen : 290 kDa

4

Type XVII collagen: 180 kDa

5

Envoplakin : 190 kDa Q/Q(M)-474641 Report a Problem

Which pair is incorrect? 5

Envoplakin : 190 kDa

Envoplakin is 210 kDa. Periplakin is 190 kDa. Q/Q(M)-474641 Report a Problem Drugs that are associated with the exacerbation of pemphigus foliaceus include : 1

Captopril

2

Metoprolol

3

Fluconazole

4

Calcium channel blockers

5

Statins Q/Q(M)-482894 Report a Problem

Drugs that are associated with the exacerbation of pemphigus foliaceus include : 1

Captopril

Pemphigus foliaceus is a form of pemphigus with superficial blist ers. The autoantibodies are against Dsg1. Medications associated with the exacerbation of pemphigus foliaceus is sulfhydryl groups such as captopril, penicillamine and piroxicam. Metroprolol can exacerbate psoriasis. Q/Q(M)-482894 Report a Problem A 60 year old gentleman presents with a recurrent pustular eruption. Biopsy demonstrates subcorneal  pustules with abundant neutrophils and a serum protein electrophoresis shows a monoclonal IgA gammopathy. The autoantigen responsible for this condition: 1

Desmoglein I

2

Desmocollin I

3

Desmoplakin

4

Plakoglobin

5

Beta 4 integrin Q/Q(M)-476599 Report a Problem

A 60 year old gentleman presents with a recurrent pustular eruption. Biopsy demonstrates subcorneal  pustules with abundant neutrophils and a serum protein electrophoresis shows a monoclonal IgA gammopathy. The autoantigen responsible for this condition: 2

Desmocollin I 7

Sneddon-Wilkenson (subcorneal pustular dermatosis) is a variant of IgA pemphigus. Clinically, patients have pustules arranged in annular and serpiginous pattern on the abdomen, axilla, and groin. The antigen is desmocollin 1 and 2. Treatment options include acitretin and dapsone. Q/Q(M)-476599 Report a Problem Patients with latex allergy aller gy are LEAST LIKELY to develop a reaction to: 1

Cashew

2

Avocado

3

Chestnut

4

Banana

5

Kiwi Q/Q(M)-474144 Report a Problem

Patients with latex allergy aller gy are LEAST LIKELY to develop a reaction to: 1

Cashew

Although rare, cross-reactivity in latex-allergic latex-all ergic patients has been demonstrated to t o banana, kiwi, avocado,  passion fruit, and chestnut. Cross-reaction to cashew has NOT been reported. Q/Q(M)-474144 Report a Problem Fiberglass dermatitis can be prevented by: 1

Water

2

Acetic acid 5%

3

Sodium chloride

4

Alkali

5

Talcum powder Q/Q(M)-474655 Report a Problem

Fiberglass dermatitis can be prevented by: 5

Talcum powder

Talcum powder application causes fiberglass spicules to slide off skin. Q/Q(M)-474655 Report a Problem

Leiner's disease (erythroderma desquamativum) is associated with: 1

C5-9 deficiency

2

Numerous infections

3

Diarrhea

4

Numerous infections and diarrhea

5

All of these answers are correct 8

Q/Q(M)-474626 Report a Problem Leiner's disease (erythroderma desquamativum) is associated with: 4

Numerous infections and diarrhea

Leiner's disease is associated with deficient C5 and possibly C3. Babies with this disease are prone to diarrhea, infections (sepsis), anemia, and a generalized seborrheic dermatitis-like rash. Q/Q(M)-474626 Report a Problem Drugs that are associated with the exacerbation of pemphigus foliaceus include : 1

Captopril

2

Metoprolol

3

Fluconazole

4

Calcium channel blockers

5

Statins Q/Q(M)-482893 Report a Problem

Drugs that are associated with the exacerbation of pemphigus foliaceus include : 1

Captopril

Pemphigus foliaceus is a form of pemphigus with superficial blist ers. The autoantibodies are against Dsg1. Medications associated with the exacerbation of pemphigus foliaceus is sulfhydryl groups such as captopril, penicillamine and piroxicam. Metroprolol can exacerbate psoriasis. Q/Q(M)-482893 Report a Problem The picture shown is considered to be diagnostic for what t ype of epidermolysis bullosa 1

Junctional epidermolysis bullosa- Non Herlitz type

2

Junctional epidermolysis bullosa- Herlitz type

3

Dowling-Meara form of Epidermolysis Bullous simplex

4

Dominant dystrophic epidermolysis bullosa

5

Recessive dystrophic epidermolysis bullosa Q/Q(M)-482116 Report a Problem

The picture shown is considered to be diagnostic for what t ype of epidermolysis bullosa 9

5

Recessive dystrophic epidermolysis bullosa

The picture is shown is know as "mitten hands" and is diagnostic for recesseive type of dystrophic epidermolysis bullosa. RDEB have the Hallopeau-Siemens type, a severe life-altering bullous disease characterized by widespread dystrophic scarring and deformity and by severe involvement of mucous membranes. Much less commonly, RDEB may manifest as the non-Hallopeau-Siemens t ypes, a mild localized form (mitis), and an inverse variant (recessive epidermolysis inversa). In the HallopeauSiemens form of RDEB, erosions and blisters are usuall y manifested at or shortly after birth. Blisters may appear to occur spontaneously, but most seem to arise at s ites of pressure or trauma. Nikolsky sign is often positive. The hands and lower aspects of the legs are particularly susceptible to severe blistering and scarring. The fingers and toes may become fused, with resultant pseudosyndactyly, in which the digits become bound together by a glove-like epidermal sac, with result ing claw-like clubbing or mittenlike deformities as shown in the image. Q/Q(M)-482116 Report a Problem Common drugs causing SJS/TEN include all except: 1

Sulfonamides

2

Carbamazepime

3

Allopurinol

4

Oxicam

5

Colchicine Q/Q(M)-474635 Report a Problem

Common drugs causing SJS/TEN include all except: 5

Colchicine

 NSAIDs, anticonvulsants, allopurinol, and sulfa drugs drugs are common inciting agents of SJS/TEN. Q/Q(M)-474635 Report a Problem Bullous pemphigoid antigen 1 (BPAg1) is a member of this family: 1

Cadherin

2

Integrin

3

Intermediate filament

4

Plakin

5

Selectin Q/Q(M)-474098 Report a Problem

Bullous pemphigoid antigen 1 (BPAg1) is a member of this family: 4

Plakin

The plakin family includes desmoplakin I and II, BPAg1, envoplakin, periplakin. Q/Q(M)-474098 Report a Problem What is the most common allergen causing allergic contact dermatitis? 1

Nickel 10

2

Bacitracin

3

Formaldehyde

4

Quaternium-15

5

Neomycin Q/Q(M)-477690 Report a Problem

What is the most common allergen causing allergic contact dermatitis? 1

Nickel

 Nickel is the most common contact allergen, found in �costume� jewelry, alloys, pigments orthopedic appliances, scissors, razors and many man y other metal coated objects. Bacitracin and neomycin are commonly used topical antibiotic agents that many are aller gic to. Neomycin is the most common topical antibiotic allergen found in testing. Quaternium-15 is a formaldehyde-releasing preservative found in many topical products. It can cross react with formaldehyde, but not all patients will react to formaldehyde on testing. While it is a common allergen, it is not the most common cause of ACD. Q/Q(M)-477690 Report a Problem The ocular form of cicatricial pemphigoid is most likely to be associated with antibodies to: 1

Beta-4-integrin

2

Laminin 5

3

BPAg1

4

BPAg2-NC16A

5

Plectin Q/Q(M)-474187 Report a Problem

The ocular form of cicatricial pemphigoid is most likely to be associated with antibodies to: 1

Beta-4-integrin

Antibodies to beta-4-integrin are associated with ocular cicatricial pemphigoid. Laminin 5 is associated a variant of cicatricial cicatricia l pemphigoid that is associated with malignancy. BPAg1 is the antigen in bullous  pemphigoid, while BPAg2-NC16A BPAg2-NC16A is associated with both bullous pemphigoid pemphigoid and herepes gestationis. Mutations in plectin are associated with epidermolysis bullosa acquisita with muscular dystrophy. Q/Q(M)-474187 Report a Problem

Mutations in beta-catenin are most commonly associated with: 1

Naxos disease

2

Bullous pemphigoid

3

Pilomatricomas

4

Ectodermal dysplasia with skin fragility

5

Ocular cicatricial pemphigoid Q/Q(M)-474184 Report a Problem

11

Mutations in beta-catenin are most commonly associated with: 3

Pilomatricomas

The armadillo family of proteins constitute of group of proteins which function in cell ular adhesion. These include beta catening, plakoglobin and plakophilins. Mutations in beta-catenin have been associated with some pilomatricdomas and colorectal carcinomas. Naxos disease has been linked to a mutation in plakoglobin, while ectodermal dysplasia with skin fragility has beenlinked to a mutation in  plakophilin 1. Ocular cicatricial pemphigoid is associated with antibodies to beta4-integrin. Q/Q(M)-474184 Report a Problem Pemphigus erythematosus: 1

Is also called Hallopeau syndrome

2

May be an abortive form of subcorneal pustulosis

3

Is often in a malar/seborrheic distribution

4

Does not have linear IgG and C3 at the basement membrane zone

5

All of these answers are correct Q/Q(M)-474636 Report a Problem

Pemphigus erythematosus: 3

Is often in a malar/seborrheic distribution

Pemphigus erythematosus, or Senear Usher syndrome, presents like pemphigus foliaceus and lupus erythematosus with very superficial erosions and erythematous/hyperkeratotic facial/chest lesions. DIF reveals intercellular IgG and linear IgG at the dermoepidermal junction. Q/Q(M)-474636 Report a Problem Which of the following is LEAST LIKELY to cross react with Toxicodendron plant dermatitis? 1

Japanese lacquer tree

2

Cashew nut

3

Mango rind

4

Gingko tree

5

Kiwi Q/Q(M)-474145 Report a Problem

Which of the following is LEAST LIKELY to cross react with Toxicodendron plant dermatitis? 5

Kiwi

The Toxicodendron group of plants is the #1 cause of contact dermatitis in North America. This group of plants includes poison ivy, i vy, poison oak, and poison sumac. Because the t he Toxicodendron plants belong to the Anacardiaceae family, cross reactions can occur with related plants and substances such as  braqzilian pepper, cashew nut, cashew oil, gingkgo tree, Indian marking nut, Japanese lacquer tree, mango, and Rengas tree. Kiwi can cross react with patients who are latex-allergic. Q/Q(M)-474145 Report a Problem

12

Patients with this disorder may develop exaggerated reactions to insect bites: 1

Chronic lymphocytic leukemia

2

Bullous pemphigoid

3

Lupus erythematosus

4

Atopic dermatitis

5

Incontinentia pigmenti Q/Q(M)-474665 Report a Problem

Patients with this disorder may develop exaggerated reactions to insect bites: 1

Chronic lymphocytic leukemia

Patients with chronic lymphocytic leukemia may develop exaggerated reactions to insect bites, including  bullous reactions. Q/Q(M)-474665 Report a Problem Pinus palustris is the source of: 1

Urea

2

D-limonene

3

Quinones

4

Lactones

5

Abietic acid Q/Q(M)-474648 Report a Problem

Pinus palustris is the source of: 5

Abietic acid

Abietic acid is the allergenic aller genic component of rosin (colophony). D-limonene is found in tea tree oil. Primin is a quinone; it is found in the primrose (Primula obconica). Abietic acid is found in rosin. Q/Q(M)-474648 Report a Problem Papillary dermal deposits of IgA and a papillary dermal infiltrate of neutrophils is diagnostic of: 1

Sweet's syndrome

2

Leukocytoclastic vasculitis

3

Dermatitis herpetiformis

4

Linear IgA dermatosis

5

Bullous pemphigoid Q/Q(M)-475854 Report a Problem

Papillary dermal deposits of IgA and a papillary dermal infiltrate of neutrophils is diagnostic of: 13

3

Dermatitis herpetiformis

Dermatitis herpetiformis is an a n uncommon chronic, pruritic papulovesicular dermatitis occurring most commonly in young to middle-aged adults. Common sites of predilection include the buttocks, elbows, knees, scapula and scalp. Typical histologic features include accumulation of neutrophils at the tips of dermal papillae, sometimes admixed with eosinophils. Direct immunoflorescence (DIF) reveals granular deposits of IgA within the dermal papillae. As the name implies, linear IgA disease is characterized by linear IgA deposition along the basement membrane zone with DIF in 100% of cases. The pattern of direct immunoflorescence in bullous pemphigoid is linear C3 deposition at the dermoepidermal junction in nearly 100% of cases and IgG in 65-95% of cases. Sweet's syndrome is not characterized by a pattern with DIF. Under light microscopy, a dense perivascular infiltrate of neutrophils is seen assuming ass uming a  bandlike pattern throughout the papillary papillary dermis associated with prominent dermal edema. Leukocytoclastic vasculitides are characterized by a perivascular neutrophilic infiltration with karyorrhexis, leukocytoclasis, fibrinoid degeneration, and endothelial swelling. Q/Q(M)-475854 Report a Problem Which of the following proteins is a 160kDa cadherin? 1

Desmoglein 3

2

Desmoglein 1

3

Desmoplakin I

4

Envoplakin

5

Plectin Q/Q(M)-474185 Report a Problem

Which of the following proteins is a 160kDa cadherin? 2

Desmoglein 1

Desmogleins and desmocollins belong to the cadherin family of proteins, which are calcium-dependent adhesion molecules. Desmoglein I is a 160kDa cadherin, while Desmoglein 3 is a 130 kDa cadherin. Desmoplakin, envoplakin, and plectin belong to the plakin family of adhesion proteins. Q/Q(M)-474185 Report a Problem Which pair is incorrect? 1

Dermatitis herpetiformis : granular IgA

2

Lichen planus : linear C3

3

Bullous pemphigoid : linear C3

4

Systemic lupus erythematosus : linear IgG

5

IgA pemphigus: intercellular IgA Q/Q(M)-474643 Report a Problem

Which pair is incorrect? 2

Lichen planus : linear C3

The colloid bodies in lichen planus can trap IgM and C3; this c an be seen on DIF as �globs.� There is no linear pattern. 14

Q/Q(M)-474643 Report a Problem What is the enzyme defect in Gunther's disease? 1

Porphobilinogen deaminase

2

Uroporphyrinogen Uroporphyrinogen synthetase III

3

Uroporphyrinogen Uroporphyrinogen decarboxylase

4

Ferrochetalase

5

Protoporphyrinogen oxidase Q/Q(M)-474593 Report a Problem

What is the enzyme defect in Gunther's disease? 2

Uroporphyrinogen synthetase III

Gunther's disease (also known as congenital erythropoietic porphyhria) is caused by a defect in uroporphyrinogen synthetase synthetase II, the enzyme enz yme which responsible for the conversion of hydroxymethylbilane to uroporphyriogen uroporphyriogen III. Patients present with red teet h and immediate  photosensitivy during infancy. There There is severe photophobia and late skin manifestations include mutilating scarswith scarring alopecia, and sclerodermoid changes. Q/Q(M)-474593 Report a Problem Cicatricial pemphigoid can be induced by: 1

Aminoglycosides

2

Benzene

3

Clonidine

4

Vancomycin

5

Ciprofloxacin Q/Q(M)-474650 Report a Problem

Cicatricial pemphigoid can be induced by: 3

Clonidine

Clonidine is a cause of drug-induced cicatricial pe mphigoid. Other common causes includee sulfa drugs and thiol-containing drugs. Q/Q(M)-474650 Report a Problem Which of the following concerning Degos' Disease is true? 1

After undergoing multiple stages, it resolves without scarring

2

It affects women more than men

3

Gatrointestinal involvement is a poor prognosis

4

Glucocorticoids are standard of treatment 15

5

Lab results indicate a low plasma fibrinogen level and decreased platelet aggregation Q/Q(M)-478696 Report a Problem

Which of the following concerning Degos' Disease is true? 3

Gatrointestinal involvement is a poor prognosis

Dego's Disease (Malignant Atrophic Papulosis): It most frequently affects men and is a potentially fatal obliterative arteritis. After undergoing multiple stages, the patient is left with varicelliform scars. Later, anemic infarcts involve the intestines to produce acute abdominal symptoms of epigastric pain, fever, and hematemesis. Death is usually due to fulminating peritonitis caused by multiple perforations of the intestines. GI involvement is a poor prognosis. Lab results indicate a high plasma fibrinogen level and increased platelet aggregation. Administration of corticosteroids has not been beneficial. Q/Q(M)-478696 Report a Problem In Brunsting-Perry pemphigoid, the recrurrent crops of blisters are most likely to appear on: 1

Genitals

2

Palms and soles

3

Umbilicus

4

Head and neck

5

Buttocks Q/Q(M)-474611 Report a Problem

In Brunsting-Perry pemphigoid, the recrurrent crops of blisters are most likely to appear on: 4

Head and neck

Brunsting-Perry pemphigoid is a variant of cicatricial pemphigoid in which there are no mucosal lesions. The most common site of blisters is the head and neck, resulting in scarring alopecia. The antigens in cicatricial pemphigoid include BPAg2 and epiligrin. Q/Q(M)-474611 Report a Problem

Which type of porphyria manifests with peripheral neuropathy, colicky abdominal pain, but NO cutaneous symptoms? 1

Acute Intermittent Porphyria

2

Gunther's disease

3

Variegate porphyria

4

Hereditary Copropophyria

5

Erythropoietic protopophyria protopophyria Q/Q(M)-474591 Report a Problem

Which type of porphyria manifests with peripheral neuropathy, colicky abdominal pain, but NO cutaneous symptoms? 1

Acute Intermittent Porphyria 16

Acute intermittent pophyria is caused by a defect in the enzyme porphobilinogen deaminase. Patients  present with peripheral neuropathy and occasionally seizures, abdominal pain, pain, and tachycardia. There are no skin changes as no dermal porphyrins are found. Q/Q(M)-474591 Report a Problem Bullous lupus erythematosus is most commonly associated with antibodies to: 1

Type XII collagen

2

Type VII collagen

3

Plectin

4

Desmoglein I

5

Laminin 5 Q/Q(M)-474201 Report a Problem

Bullous lupus erythematosus is most commonly associated with antibodies to: 2

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. The The histology can resemble that of dermatitis herpetiformis and DIF shows linear IgG, IgM, IgA and C3. Q/Q(M)-474201 Report a Problem What is the most common site of involvement of this autoimmune blistering disease? 1

Oral mucosa

2

Eyes

3

Skin

4

Genitalia

5

Nasopharyngeal mucosa Q/Q(M)-476549 Report a Problem

What is the most common site of involvement of this autoimmune blistering disease? 1

Oral mucosa

Cicatricial pemphigoid is a heterogeneous group of diseases with chara cteristic involvement of the oral mucosa, eyes, skin. Rarely, it can also involve the nasopharyngeal mucosa, external genitalia, esophagus, and anus. The oral mucosa is the most common site of involvement. Q/Q(M)-476549 Report a Problem 17

Which of the following substances is known to cause a delayed positive patch test reaction? 1

Gold

2

Nickel

3

Bacitracin

4

Fragrance mix

5

Rosin Q/Q(M)-482259 Report a Problem

Which of the following substances is known to cause a delayed positive patch test reaction? 1

Gold

Gold is known to cause a delayed patch test reading. Patients allergic to gold often also react to nickel and cobalt. Bacitracin is a frequent contact allergen that often coexists with an allergy to neomycin. Fragrance mix contains cinnamic alcohol alcohol and aldehyde, hydroxycitronellel, isoeugenol, eugenol, oak moss moss absolute, alpha-amyl cinnamic aldehyde, geraniol. geraniol. Rosin (colophony) is found in adhesive tape, cosmetics, glossy papers, violin bows, and chewing gum. Q/Q(M)-482259 Report a Problem The antigen in linear IgA is: 1

BPAg1

2

Plectin

3

97kDa part of BPAg2

4

Laminin 5

5

Alpha-6, beta integrin Q/Q(M)-474607 Report a Problem

The antigen in linear IgA is: 3

97kDa part of BPAg2

The antigen in linear IgA and its variant, chronic bullous diseas e of childhood, is the 97kDa part of BPAg2. BPAg1 is the antigen in bullous pemphigoid. Plectin is defective in EB simplex with muscular dystrophy. Laminin 5 is defective in junctional EB and alpha 6, beta integrin is defective i n junctional EB with pyloric atresia. Q/Q(M)-474607 Report a Problem Th2-related cytokines include: 1

IL10

2

IFNgamma

3

IFNalpha

4

IL10 and IFNgamma

5

All of these answers are correct 18

Q/Q(M)-474627 Report a Problem

Th2-related cytokines include: 1

IL10

Atopic dermatitis is considered a disease in which a Th2 pattern predominates, especially early on. Th2 cytokines are IL4, IL5, IL6, IL10, I L10, IL13. Q/Q(M)-474627 Report a Problem The epidermolysis bullosa acquisita antigen is: 1

A transmembrane protein

2

An intracellular protein

3

A desmosomal protein

4

Located in the lamina lucida

5

An anchoring fibril Q/Q(M)-476546 Report a Problem

The epidermolysis bullosa acquisita antigen is: 5

An anchoring fibril

Epidermolysis bullosa acquisita is a rare autoimmune disorder with an estimated incidence of 0.25 per million. In the classic form, it i t is characterized by non-inflammatory bulla with a prediliction for traumatized sites. The autoantigen is t ype VII collagen, which is an anchoring fibril located in the sublamina densa. Following salt split skin, the type VII collagen can be localized at the base. Q/Q(M)-476546 Report a Problem A child has a defect in type VII collagen and presents with acral blisters which heal with milia and scarring. What is the diagnosis? 1

Albopapuloid/Pasini variant of dystrophic epidermolysis bullosa

2

Bart's syndrome

3

Cockayne-Touraine disease

4

Epidermolysis bullosa acquisita

5

Weber-Cockayne Q/Q(M)-482367 Report a Problem

A child has a defect in type VII collagen and presents with acral blisters which heal with milia and scarring. What is the diagnosis? 3

Cockayne-Touraine disease

This patient has Cockayne-Touraine disease which is c haracterized by a defect in type t ype VII collagen and  presents with acral blisters which heal with milia and scarring. The albopapuloid variant of dystrophic dystrophic epidermolysis bullosa presents with flesh-colored, scar-like papules and dystrohpic nails. Bart's syndrome is associated with aplasia cutis congenita usually on the extremities. Weber-Cockayne does 19

have acral lesions but it is associated with a defect in keratins 5 and 14. The pathogenesis of epidermolysis bullosa acquisita involves autoimmunity to collagen VII, not a defect in it. Q/Q(M)-482367 Report a Problem Which of the following is true of herpes gestationis? 1

Demonstrates antibodies to the C-terminal domain of BPAg2

2

Has higher frequency in females with HLA-DQ2

3

Is associated with Grave's disease

4

Usually occurs in the first trimester of pregnancy

5

Shows subepidermal bullae mostly with neutrophils Q/Q(M)-482366 Report a Problem

Which of the following is true of herpes gestationis? 3

Is associated with Grave's disease

Herpes gestationis is associated with Grave's disease. It is more common with patients with HLA-DR3 and DR4 and most often occurs between 4-7 months of pregnancy. The antibodies are against the NC 16-A portion of BPAg2. Histology shows subepidermal bullae with mostly eosinophils. Q/Q(M)-482366 Report a Problem A 59-year old female has erythema and vesiculation in an area of application of tincture of benzoin. Which allergen is she also most likely allergic to? 1

Ammonium persulfate

2

Black rubber mix

3

Balsam of Peru

4

Eugenol

5

Epoxy resin Q/Q(M)-482267 Report a Problem

A 59-year old female has erythema and vesiculation in an area of application of tincture of benzoin. Which allergen is she also most likely allergic to? 3

Balsam of Peru

This patient who has a contact dermatitis to tincture of benzoin is probably reacting to its allergenic ingredient Balsalm of Peru which has healing and antibacterial properties. Ammonium persulfate is a  bleaching agent for hair and flour, not to be confused with ammonium thioglycolate, a perming agent. agent. Black rubber mix can produce unique lichenoid and purpuric reactions. Eugenol is found in cloves. Epoxy resin is found in glues and plastics. Q/Q(M)-482267 Report a Problem Which of the associated antigens for this condition has been associated with colon cancer? 1

Anti-epiligrin

2

97 kD linear IgA dermatosis antigen

3

Desmoplakin 20

4

Bullous pemphigoid antigen 2

5

Beta-4 integrin Q/Q(M)-476551 Report a Problem

Which of the associated antigens for this condition has been associated with colon cancer? 1

Anti-epiligrin

While the oral mucosa is the most common site for cicatricial pemphigoid, ocular involvement is very common. Clinical manifestations may include shortened fornices, symblephera, ankyloblephera. The majority of of ocular antigens are thought to reside in t he cytoplasmic domain of beta-4 integrin. Q/Q(M)-476551 Report a Problem Bullous lesions are seen in: 1

Congenital syphilis

2

Primary syphilis

3

Secondary syphilis

4

Tertiary syphilis

5

All of these answers are correct

Q/Q(M)-474074 Report a Problem Bullous lesions are seen in: 1

Congenital syphilis

Congenital syphilis, but not other forms of syphilis, can occasionally present with bullae. Q/Q(M)-474074 Report a Problem A 35 year-old dentist presents with tingling in his fingertips. What allergen is most likely to cause this dermatitis? 1

Methyl methacrylate

2

Paraphenylenediamine

3

Colophony

4

Thiuram mix

5

Ethylenediamine dihydrochloride Q/Q(M)-476779 Report a Problem

21

A 35 year-old dentist presents with tingling in his fingertips. What allergen is most likely to cause this dermatitis? 1

Methyl methacrylate

Methyl methacrylate is found in synthetic resins, dentures, artificial, nail adhesives, and acrylic bone cement. This allergen may penetrate through the gloves to t he fingertips and most often affects the first f irst three fingers. In addition to dermatitis, it also causes a peripheral neuropathy. Q/Q(M)-476779 Report a Problem At what level is the blister separation plane in linear IgA dermatosis? 1

Granular layer

2

Suprabasal layer

3

Basal layer

4

Lamina lucida

5

Lamina densa Q/Q(M)-477133 Report a Problem

At what level is the blister separation plane in linear IgA dermatosis? 4

Lamina lucida

The cleavage in linear IgA dermatosis (LAD) ( LAD) typically occurs in the lamina lucida as the target antigen is the 97 kD cleaved portion of bullous pemphigus antigen II (BPAgII). A variant of LAD may separate in the sublamina densa, with a target antigen of the type VII collagen in the anchoring fibrils. This condition may also be induced by ingestion of certain medications including vancomycin, lithium and diclofenac. Q/Q(M)-477133 Report a Problem

Which neoplasm is the most common cause of paraneoplastic pemphigus? 1

Thymoma

2

CLL

3

Castleman's disease

4

Retroperitoneal sarcoma

5

Non-Hodgkin's lymphoma Q/Q(M)-477484 Report a Problem

Which neoplasm is the most common cause of paraneoplastic pemphigus? 5

Non-Hodgkin's lymphoma

All of the above have been associated with paraneoplasti c pemphigus with non-Hodgkin's lymphoma  being the most common. Castlemans's disease is most common in children with paraneoplastic paraneoplastic  pemphigus. 22

Q/Q(M)-477484 Report a Problem Irritation of the hand produced by capsaicin can be relieved by application of 1

Water

2

Acetic acid 5%

3

Sodium chloride

4

Alkali

5

Talcum powder Q/Q(M)-474654 Report a Problem

Irritation of the hand produced by capsaicin can be relieved by application of 2

Acetic acid 5%

Burning secondary to capsaicin can be relieved by vinegar ( acetic acid 5%) as the capsaicin c apsaicin is soluble in vinegar (but not water). Q/Q(M)-474654 Report a Problem Which of the following contains the same allergen as poison sumac? 1

Mango fruit

2

Brazilian Pepper Tree

3

Balsam of Peru

4

Ragweed

5

Artichoke Q/Q(M)-482457 Report a Problem

Which of the following contains the same allergen as poison sumac? 2

Brazilian Pepper Tree

The rhus family (poison ivy, poison oak, poison sumac) contain urushiol (a pentadecacatechol). This allergen is also associated associate d with contact allergy to Cashew nut, Japanese laquer tree, Mango rind (not fruit), Gingko, Brazilian pepper tree, Pepeo tree of Venezuela, El litre tree of Chile and the Rengas (black varnish) tree. Q/Q(M)-482457 Report a Problem Circulating autoantibodies to type XVII collagen are most characteristic of which disease? 1

Epidermolysis bullosa accquisita

2

Herpes gestationis

3

Pemphigus vulgaris

4

Pemphigus foliacious

5

Paraneoplastic pemphigus Q/Q(M)-476544 Report a Problem 23

Circulating autoantibodies to type XVII collagen are most characteristic of which disease? 2

Herpes gestationis

Type XVII collagen or BPAG2 is a transmembrane protein found in the hemidesmosome. Autoantibodies to type XVII collagen are important in the pathogenesis of cicatricial pemphigoid,  bullous pemphigoid, and herpes herpes gestationis. Q/Q(M)-476544 Report a Problem Which pair is incorrect? 1

Wrinkle-resistance : formaldehyde

2

Chewing gum : colophony

3

Parabens : artichokes

4

Glyceryl thioglycolate : acid permanent wave

5

Permethrin : chrysanthemum Q/Q(M)-474649 Report a Problem

Which pair is incorrect? 3

Parabens : artichokes

Wrinkle-resistant clothing is treated with formaldehyde. Chewing gum contains rosin (colophony). Parabens are preservatives. Artichokes are a member of the family Asteraceae (formerly Compositae). Glyceryl thioglycolate and methylchloroisothiazolinone are found in acid permanent wave products. Permethrins are synthetic pyrethroids that are similar to pyrethrins derived from the Asteraceae family. Permethrins can cross-react with allergy to chrysanthemums. Q/Q(M)-474649 Report a Problem

Paraneoplastic pemphigus: 1

Is characterized by a pathognomonic 250 kDa antigen

2

Is most often seen in association with lung cancer

3

Does not remit even if the cancer is excised completely

4

May be caused by a benign neoplasm

5

All of these answers are correct Q/Q(M)-474637 Report a Problem

Paraneoplastic pemphigus: 4

May be caused by a benign neoplasm

Paraneoplastic pemphigus can be caused by lymphoma as well as other ma lignancies. It can also be secondary to benign thymomas and Castleman's disease. Numerous antigens have been found, including desmoplakin I and II. Desmoplakin I and II may be antigenic in Stevens-J ohnson syndrome. Q/Q(M)-474637 Report a Problem Gallstones are associated with: 24

1

Erythropoietic protoporphyria

2

Hepatoerythropoietic porphyria

3

Variegate porphyria

4

Coproporphyria

5

Harderoporphyria Q/Q(M)-474102 Report a Problem

Gallstones are associated with: 1

Erythropoietic protoporphyria

Gallstones and liver failure can complicate erythropoietic protoporphyria. Q/Q(M)-474102 Report a Problem Ocular cicatricial pemphigoid has antibodies against: 1

Gamma-catenin

2

Peripherin

3

Beta4-integrin

4

Kalinin

5

Vinculin Q/Q(M)-474657 Report a Problem

Ocular cicatricial pemphigoid has antibodies against: 3

Beta4-integrin

Ocular cicatricial pemphigoid (CP) has autoantibodies against beta4-integrin. CP associated with malignancy has autoantibodies against epiligrin (laminin 5). CP with mucous membrane and skin findings often has autoantibodies against BPAg2. Q/Q(M)-474657 Report a Problem The most common malignancy associated with paraneoplastic pemphigus is: 1

Non-Hodgkin's lymphoma

2

Chronic lymphocytic leukemia

3

Multiple myeloma

4

Acute myelocytic leukemia

5

Hodgkin's lymphoma Q/Q(M)-474101 Report a Problem

The most common malignancy associated with paraneoplastic pemphigus is: 1

Non-Hodgkin's lymphoma

Paraneoplastic pemphigus is associated with various benign as well as malignant internal tumors, with the most common being non-Hodgkin's lymphoma. 25

Q/Q(M)-474101 Report a Problem Which of the following is not a formaldehyde-releasing preservative? 1

Bronopol

2

Methylchloroisothiazinolone

3

Quaternium-15

4

Imidazolidinyl urea

5

DMDM hydantoin Q/Q(M)-482258 Report a Problem

Which of the following is not a formaldehyde-releasing preservative? 2

Methylchloroisothiazinolone

Methylchloroisothiazinolone, also known as Kathon CG, is the only non-formaldehyde releasing  preservative. It is found in cosmetics, skin/hair products, "acid" permanent waves, soaps, latex emulsions, and biocides. All the others others are formaldehyde-releasing preservatives. Q/Q(M)-482258 Report a Problem Patients with Senear-Usher syndrome are most likely to present with: 1

Erythematous papules and plaques around the umbilicus

2

Transient vesicles on the oral mucosa

3

Severely pruritic grouped vesicles symmetrically distributed primarily on extensor surfaces

4

Erythema multiforme-like oral ulcerations which are severe

5

Erythematous crusts and hyperkeratotic lesions on the nose, ears, cheeks, scalp, and chest Q/Q(M)-474191 Report a Problem

Patients with Senear-Usher syndrome are most likely to present with: 5

Erythematous crusts and hyperkeratotic lesions on the nose, ears, cheeks, scalp, and chest

Senear-Usher syndrome, also known as pemphigus erythematosus, is thought to be an overlap between lupus erythematosus and pemphigus foliaceous. The histology resembles that of pemphigus foliaceous with linear IgG at the dermoepidermal junction. 80% of patiens have a positive lupus band. Q/Q(M)-474191 Report a Problem The vector of fogo selvagem may be: 1

Triatoma

2

Simulium

3

Cimex

4

Ornithodorus

5

Mus Q/Q(M)-474661 Report a Problem 26

The vector of fogo selvagem may be: 2

Simulium

Fogo selvagem is considered to be an endemic form of pemphigus foliaceus, firs t described in Brazil, in rural areas where Simulium (black fly) fl y) is often found. Subsequently, other rural South American areas have had clusters of affected patients. Q/Q(M)-474661 Report a Problem Each of the following is true about anti-p200 pemphigoid except: 1

Responsive to dapsone

2

Subepidermal bullae

3

200-kd antigen

4

features of linear IgA disease

5

mucosal involvement Q/Q(M)-477316 Report a Problem

Each of the following is true about anti-p200 pemphigoid except: 5

mucosal involvement

Anti-p200 pemphigoid is an autoimmune blistering disorder. These autoantibodies bind to a 200-kD antigen found at the lamina lucida-lamina densa interface, thereby causing a subepidermal bullae. Clinical features of this disease are similar to bullous pemphigoid, dermatitis herpetiformis and linear IgA disease. In the case reported r eported by Egan, et.al., the patient responded well to steroids and dapsone and did not have mucosal lesions. Q/Q(M)-477316 Report a Problem A patient develops erythema and vesiculation on his dorsal foot that spares the toewebs and soles after  buying a new pair of shoes. What is the most likely allergen? 1

Thiuram mix

2

Carbamates

3

Latex

4

Mercaptobenzothiazole

5

Formaldehydge Q/Q(M)-482380 Report a Problem

A patient develops erythema and vesiculation on his dorsal foot that spares the toewebs and soles after  buying a new pair of shoes. What is the most likely allergen? 4

Mercaptobenzothiazole

This patient has allergic shoe dermatitis. The number one cause is mercaptobenzothiazole, a rubber accelerator. Other rubber accelerators include carbamates--found in bleached fabrics like waistbands and consumer rubber products such as condoms, swimwear etc--and thiuram mix, found in almost all rubber. Of note thiuram is also present in antiscabies medication and the disulfiram. 27

Q/Q(M)-482380 Report a Problem Patients with Duhring's disease are most likely to have: 1

Mutations in plectin

2

Mutations in laminin 5

3

Mutations in transglutaminase I

4

Antibodies to transglutaminase 3

5

Antibodies to BPAg2 Q/Q(M)-474188 Report a Problem

Patients with Duhring's disease are most likely to have: 4

Antibodies to transglutaminase 3

Duhring's disease is also known as dermatitis herpetiformis. Antibodies are found to transglutaminase 3, and the direct immunofluorescent studies show granular IgA and C3 in the dermal papillae. Mutati ons in  plectin are found in EBS with muscular dystrophy. dystrophy. Mutations in laminin 5 are found found in patients with JEB,Herlitz type. Mutations in transglutaminase I are found in pateints with lamellar ichthyosis and non  bullous congenital ichthyosiform erythroderma. Q/Q(M)-474188 Report a Problem

Common causes of drug-induced linear IgA: 1

Vancomycin

2

Penicillin

3

Cephalosporins

4

Captopril

5

All of these answers are correct Q/Q(M)-474633 Report a Problem

Common causes of drug-induced linear IgA: 5

All of these answers are correct

Linear IgA disease that is induced by drugs is most commonly secondary to vancomycin. Other implicated drugs include other antibiotics and captopril. Q/Q(M)-474633 Report a Problem Which association is incorrect? 1

Epidermolysis bullosa acquisita : inflammatory bowel disease

2

Dermatitis herpetiformis : small bowel lymphoma

3

Paraneoplastic pemphigus : Castleman’s

4

Herpes gestationis : menopause

5

Porphyria cutanea tarda : hemochromatosis Q/Q(M)-474639 Report a Problem 28

Which association is incorrect? 4

Herpes gestationis : menopause

Herpes gestationis is an immunoblistering disease of pregnancy. Unlike pruritic urticarial papules and  plaques of pregnancy, it recurs with successive pregnancies. It is associated with Grave's disease and choriocarcinoma. Q/Q(M)-474639 Report a Problem The C-terminal domain of BPAg2 is targeted in: 1

Bullous pemphigoid

2

Cicatricial pemphigoid

3

IgA pemphigus

4

Duhring�s disease

5

Pemphigoid gestationis Q/Q(M)-474664 Report a Problem

The C-terminal domain of BPAg2 is targeted in: 2

Cicatricial pemphigoid

The C-terminal domain of BPAg2 is targeted in cicatricial pemphigoid. The NC16A domain of BPAg2 is targeted in bullous pemphigoid. Q/Q(M)-474664 Report a Problem Patients with pemphigus vulgaris have lesions that start in the mouth in approximately : 1

70% of patients

2

50% of patients

3

30% of patients

4

20% of patients

5

10% of patients Q/Q(M)-482892 Report a Problem

Patients with pemphigus vulgaris have lesions that start in the mouth in approximately : 1

70% of patients

In 70% of patients, pemphigus vulgaris starts in the mouth and other mucosal surfaces can also be involved. Q/Q(M)-482892 Report a Problem You are consulted on this patient who has just undergone an orthopedic spinal procedure. What is true of this patient? 1

She should avoid bananas 29

2

She is allergic to betadine

3

She is most likely having an allergic response to an antibiotic

4

She has miliaria crystallina

5

This is not related to her procedure Q/Q(M)-480562 Report a Problem

You are consulted on this patient who has just undergone an orthopedic spinal procedure. What is true of this patient? 1

She should avoid bananas

This patient had an allergic contact dermatitis dermati tis to latex products used during the procedure. Latex cross reacts with bananas, avocados, kiwis and chestnuts. The linear and geometric distribution is a clue to the t he diagnosis. Q/Q(M)-480562 Report a Problem

"Oriental" tiger balm cross-reacts with: 1

Balsam of Peru

2

Ylang-Ylang oil

3

Neomycin

4

Lanolin

5

Rosin Q/Q(M)-474146 Report a Problem

"Oriental" tiger balm cross-reacts with: 1

Balsam of Peru

"Oriental" tiger balm is a popular Chinese proprietary ointment used to relieve aches a nd pains. Patients who develop allergic contact dermatitis to this agent have also been shown to have cross-reactions with  balsam of Peru. A similar substance known as "Bavarian" tiger balm has been reported to cross cross react with rosin. Q/Q(M)-474146 Report a Problem Direct immunofluorescent studies in a patient with bullous pemphigoid is most likely to show: 30

1

Linear IgA at the basement membrane

2

Linear C3 at the basemement membrane

3

Granular IgA in dermal papillae

4

Intercellular IgG4 throughout the epidermis

5

C3 in the dermal papillae Q/Q(M)-474186 Report a Problem

Direct immunofluorescent studies in a patient with bullous pemphigoid is most likely to show: 2

Linear C3 at the basemement membrane

In bullous pemphigoid, the antigenic targets are believed to be BPAg1 and BPAg2-NC16A domain. These proteins are located in the hemidesmosome. Direct immunofluorescent studies reveal linear  basement membrane C3in approximately 95% of patients, and IgG4 in approximately 80%. Linear Linear IgA is found in linear IgA and chronic bullous deiseasaeof childhood. Granular IgA and C3 in the dermal  papillae is found in dermatitits herpetiformis. Intercellular IgG4 throughout the epidermis is found in  pemphigus vulgaris. Q/Q(M)-474186 Report a Problem

Which autoantibodies would most likely likel y be found in an individual with eye findings without any cutaneous involvement? 1

Alpha 6 beta 4 integrin

2

Bullous pemphigoid antigen 1

3

Bullous pemphigoid antigen 2

4

Desmocollin

5

Laminin 5 Q/Q(M)-476541 Report a Problem

Which autoantibodies would most likely likel y be found in an individual with eye findings without any cutaneous involvement? 1

Alpha 6 beta 4 integrin

Cicatricial pemphigoid is a rare, heterogeneous hete rogeneous group of blistering diseases with predilection for mucous membranes. It may involve the eyes, oral mucosa, genital/anal mucosa, and s kin. The ocular form of cicatricial pemphigoid without cutaneous findings is usually associated with circulating autoantibodes to alpha 6 beta 4 integrin. Q/Q(M)-476541 Report a Problem

Which of the following is associated with dermatitis herpetiformis? 1

Gluten-sensitive enteropathy

2

Inflammatory bowel disease

3

Autoimmune hepatitis 31

4

Herpes labialis

5

Rheumatoid arthritis Q/Q(M)-482818 Report a Problem

Which of the following is associated with dermatitis herpetiformis? 1

Gluten-sensitive enteropathy

Gluten-sensitive enteropathy or Celiac disease is demonstrated on small bowel biopsy of all patients with dermatitis herpetiformis. However, most of these patients do not demonstrate symptoms of gastrointestinal disease. Dermatitis herpetiformis (DH) is not associated with inflammatory bowel disease, autoimmune hepatitis, herpes labialis, or rheumatoid arthritis. Q/Q(M)-482818 Report a Problem Epidermolysis bullosa simplex is caused by mutations in: 1

Keratins 1&10

2

Keratins 1&9

3

Keratins 5&14

4

Keratins 6a &16

5

Keratins K2e&10 Q/Q(M)-474483 Report a Problem

Epidermolysis bullosa simplex is caused by mutations in: 3

Keratins 5&14

EB Simplex is caused by mutations in keratins 5 &14. Mutations in Keratins 1&10 are associated with epidermolytic hyperkeratosis ande Unna-thost. Mutations in keratins 1&9 are asso ciated with epidermolytic palmoplantar keratoderma. Mutations in Keratins 6a and 16 are associated with Pachyonychia congenital. Mutations in K2e and 10 are associated with Icthyosis bullosa of Siemens. Q/Q(M)-474483 Report a Problem Which of the following is the most common cause of contact dermatitis due to a formaldehyde releasing  preservative? 1

Paraben mix

2

Thimerosal

3

Quaternium 15

4

Imidazolidinyl urea

5

Paraphenylenediamine Q/Q(M)-474138 Report a Problem

Which of the following is the most common cause of contact dermatitis due to a formaldehyde releasing  preservative? 3

Quaternium 15 32

Quaternium 15 is the primary cause of contact dermatitis among formaldehy-releasing preservatives. Other formaldehyde-releasing preservatives include imidazolidinyl urea, diazolidinyl urea, DMDM hydantoin, Bronopol, Cyanobutane, Ethyleneurea melamine formaldehy resin (found in permanent press clothing), and dimethylol dihydroxyethyleneurea. Paraben mix (#1 preservative in topicals), thimerosal (a type of mercurial antiseptic), and paraphenylenediamine (hair dye) are non-formaldehyde releasing  preservatives. Q/Q(M)-474138 Report a Problem A 20-year-old female presents with an allergic contact dermatitis to a perfume containing Lily of the valley. What is the causative allergen? 1

Cinnamic acid

2

Vanillin

3

Hydroxycitronellal

4

Atranorin

5

Evernic acid Q/Q(M)-482378 Report a Problem

A 20-year-old female presents with an allergic contact dermatitis to a perfume containing Lily of the valley. What is the causative allergen? 3

Hydroxycitronellal

The main allergen in Lily of the valley, valle y, which is found in perfumes, soaps, cosmetics, eye cream and aftershaves, is hydroxycitronellal (synthetic). Cinnamic acid and vanillin are present in Balsam of Peru, while atranorin and evernic acid are allergens in oak moss absolute. Q/Q(M)-482378 Report a Problem Cleavage of this adhesion molecule is seen in Staphylococcal Scalded Skin Syndrome: 1

Desmoplakin 1

2

Desmoplakin 2

3

Desmoglein 1

4

Desmoglein 3

5

Desmoplakin 1 and desmoplakin 2 Q/Q(M)-474624 Report a Problem

Cleavage of this adhesion molecule is seen in Staphylococcal Scalded Skin Syndrome: 3

Desmoglein 1

Desmoglein 1 is cleaved by exfoliative toxin in Staphylococcal scalded skin syndrome. Q/Q(M)-474624 Report a Problem This is associated with deafness: 1

Claudin

2

Cadherin 33

3

Connexin

4

Desmin

5

Desmoplakin Q/Q(M)-474659 Report a Problem

This is associated with deafness: 3

Connexin

Connexin 26 is associated with PPK and deafness. Mutations in this same connexin is associated with Vohwinkel�s and KID syndromes. Q/Q(M)-474659 Report a Problem Which form of epidermolysis bullosa presents with generalized bullae, absent nails, dysplastic teeth (due to enamel defects), nonhealing granulation tissue periorally, and is often fatal by age 3-4? 1

Weber-cockayne

2

Herlitz

3

Non-Herlitz

4

Hyperplastic cockayne-touraine

5

Albapapuloid Pasini variant Q/Q(M)-474485 Report a Problem

Which form of epidermolysis bullosa presents with generalized bullae, absent nails, dysplastic teeth (due to enamel defects), nonhealing granulation tissue periorally, and is often fatal by age 3-4? 2

Herlitz

Junctional EB is an autosomal recessive disorder with a defect in the lamina lujcida; uncein is absent in all forms. The Herlitz variant is due to mutations in laminin 5 and clinically presents as generalized  bullae (which are non-scarring), nonhealing granulation tissue periorally, absent nails, dysplastic teeth and enamel defects, anemia, growth retardation and is often fatal by age 3-4 due to low protein, anemia and infection. The non-Herlitz variant of Junctional EB presents with bullae especially on the extremities which heal with atrophic scarring; pts improve with age and typically have a normal life span. Weber-cockayne is a type of EB simplex and presents with palmoplantar bullae and hyperhidrosis. Hyperplastic cockayne-touraine is a form of dominant dystrophic EB which presents with bullae localized to extremities. Albapapuloid Pasini variant of dystropohic EB presents with widespread bullae, atrophy, hypopigmented scar-like white papules on trunk, no miliaar or scarring, and mild oral involvement. Q/Q(M)-474485 Report a Problem Which of the following can be responsible for contact dermatitis to K-Y Jelly? 1

Lanolin alcohol

2

Budesonide

3

Alpha tocopherol

4

Propylene glycol

5

Triclosan 34

Q/Q(M)-474142 Report a Problem Which of the following can be responsible for contact dermatitis to K-Y Jelly? 4

Propylene glycol

Propylene glycol is a widely used solvent and humectant found in a variety of products such as cosmetics, lotions, corticosteroids, antiperspirants, and K-Y jelly. Lanolin is an emollient which comes from wool wax and is found in adhesives, cosmetics, and topical emollients such as Aquaphor. Budesonide is a steroid, alpha tocopherol is topical Vitamin E, and Triclosan is a topical antibiotic. Q/Q(M)-474142 Report a Problem

Clumped tonofilaments are seen on electron microscopy in: 1

Dowling-Meara

2

Weber-Cockayne

3

Gunther �s

4

Naxos disease

5

Vohwinkel�s syndrome Q/Q(M)-474660 Report a Problem

Clumped tonofilaments are seen on electron microscopy in: 1

Dowling-Meara

Clumped tonofilaments (keratin intermediate filaments) are seen in EB simplex subtype s ubtype DowlingMeara. Weber-Cockayne is a subtype of EB simplex with t rauma-induced blistering on the soles of the feet and occasionally palms. Q/Q(M)-474660 Report a Problem Bullous diabeticorum typically presents on the: 1

Face

2

Chest

3

Arms

4

Legs

5

Groin Q/Q(M)-474596 Report a Problem

Bullous diabeticorum typically presents on the: 4

Legs

Bullous diabetocorum presents as tense blisters on the legs. They are typically non-inflammatory and appear suddenly. Q/Q(M)-474596 Report a Problem The defect in junctional epidermolysis bullosa occurs at the:

35

1

Granular layer

2

Spinous layer

3

Lamina lucida

4

Lamina densa

5

Sublamina densa Q/Q(M)-474605 Report a Problem

The defect in junctional epidermolysis bullosa occurs at the: 3

Lamina lucida

Junctional epidermolysis bullosa is an autosomal recessive disorder characterized by a defect in the lamina lucida. The protein uncein, a molecule which binds the gamma chain of laminin and a6b4/BPAg1, is absent in all forms of junctional EB. Uncein is normally found in the lamina lucida, along with lamin 1, laminin 5, laminin 6m, heparan sulfate proteoglycan, and fibronectin. Q/Q(M)-474605 Report a Problem Epidermolysis bullosa simplex with muscular dystrophy is associated with a mutation in: 1

Laminin 5

2

Plectin

3

collagen VII

4

uncein

5

alpha 6 beta integrin Q/Q(M)-474484 Report a Problem

Epidermolysis bullosa simplex with muscular dystrophy is associated with a mutation in: 2

Plectin

EB Simplex is an autosomal dominant disorder caused by mutations in keratins 5 &14, leading to intraepidermal bullae. A subtype of EBS patients have muscular d ystrophy due to a defect in plectin. Laminin 5 mutations are associated with junctional EB. Collagen VII mutations are associated with dystrophic EB. Uncein is a protein in the lamina lucida which is found to be absent in junctional EB. Mutations in alpha6-beta4 integrins are associated with JEB with pyloric atresia. Q/Q(M)-474484 Report a Problem Which pair is incorrect? 1

Eyedrops : thimerosal

2

Ear drops : neomycin

3

Theophylline : ethylenediamine

4

Antabuse : thiuram

5

Cobalamin : balsam Q/Q(M)-474645 Report a Problem 36

Which pair is incorrect? 5

Cobalamin : balsam

Cobalamin is Vitamin B12; cobalt is a component of Vitamin B12. Balsam of Peru is found in fragrances. Q/Q(M)-474645 Report a Problem Herpes gestationis is most commonly associated with: 1

Grave's

2

Hashimoto's

3

Diabetes

4

Lupus

5

Rheumatoid arthritis Q/Q(M)-474629 Report a Problem

Herpes gestationis is most commonly associated with: 1

Grave's

Autoimmune diseases are associated with herpes gestationis; Grave's disease is the most common association. Q/Q(M)-474629 Report a Problem What is the antigen associated with dermatitis herpetiformis? 1

Tissue transglutaminase

2

Epilegrin

3

Desmocollin

4

Desmoglein 3

5

Periplakin Q/Q(M)-476598 Report a Problem

What is the antigen associated with dermatitis herpetiformis? 1

Tissue transglutaminase

Dermatitis herpetiformis is an autoimmune blistering disease associated with intense pruritus. Classically, it involves the extensor aspects of the body. Antibodies to tissue transglutaminase (antiendomesial, anti-gliadin, anti-reticulin) can be found. It is a ssociated with celiac sprue. 50% have thyroid disease. Q/Q(M)-476598 Report a Problem Gap junctions consist of: 1

Laminin

2

Plectin

3

Uncein 37

4

Filagrin

5

Connexins Q/Q(M)-474602 Report a Problem

Gap junctions consist of: 5

Connexins

Gap junctions are protein channels that allow for communcation between cells. A connexon of one cell is joined to that of an adjacent cell to form an intercellular channel consisting of 12 connexin subunits. Each intercellular channel provides a channel that interconnects the cytoplasm of the apposed cell directly and permits the passage of ion and other s mall molecules between adjacent cells. These protein channels that make up the gap junctions consist of two hemi -channels or connexons. One connexon resides in the membrane of one cell and it aligns and joins the connexon of the neighboring cell, forming a continuous aquaeous pathway by which these ions and small molecules can freely pass from one cell to the other. Each hemi-channel or connexon consist of six proteins ( hexamer) called connexins. Q/Q(M)-474602 Report a Problem In penicillamine-induced pemphigus, the split is most often: 1

Subcorneal

2

Intraspinous

3

Suprabasal

4

Intraepidermal and subepidermal

5

Subepidermal Q/Q(M)-474651 Report a Problem

In penicillamine-induced pemphigus, the split is most often: 1

Subcorneal

Penicillamine is the most common cause of drug-induced pemphigus, and the split is more often subcorneal (pemphigus foliaceus-like) than suprabasal (pemphigus vulgaris-like). Q/Q(M)-474651 Report a Problem A one-day old infant presents with papules and pustules on the face, t runk, and proximal extremities. Palms and soles are spared. The patient is otherwise doing well. A biopsy shows numerous eosinophils. The diagnosis is: 1

Acropustulosis of infancy

2

Herpes infection

3

Erythema toxicum neanotorum

4

Scabies

5

Transient neonatal pustular melanosis Q/Q(M)-474595 Report a Problem

A one-day old infant presents with papules and pustules on the face, t runk, and proximal extremities. Palms and soles are spared. The patient is otherwise doing well. A biopsy shows numerous eosinophils. The diagnosis is: 38

3

Erythema toxicum neanotorum

Papule and pustules on an erythematous base appear on a health y infant within 48 hours, and typically last 2-3 days in erythema toxicum neanotorum. The lesions may occur anywhere, but usually spare the  palms and soles. Gram stain reveals numerous eosinophils. A 1 day-old infant would not have had a chance to acquire a scabies infestation. Acropustulosis of infancy typically presents at 3 -4 months and the pathology shows vesicles with many neutrophils. A herpes infection would not show eosinophils. Transient neonatal pustular melanosis shows sterile sub corneal pustules. Q/Q(M)-474595 Report a Problem Which of the following adhesion molecules are important for maintaining adhesion between keratinocytes? 1

Selectins

2

Integrins

3

Cadherins

4

Glycans

5

Immunoglobulin superfamily Q/Q(M)-477148 Report a Problem

Which of the following adhesion molecules are important for maintaining adhesion between keratinocytes? 3

Cadherins

Cadherins mediate cell adhesion and play a fundamental role in normal development. Calicum is required for the normal function of these adhesion molecules. There are two main subclasses, classic class ic (E, P-, N-) and desmosomal (desmoglein and desmocollin). Q/Q(M)-477148 Report a Problem Which of the following is true of a phototoxic reaction? 1

Requires prior sensitization of photoallergen in susceptible individuals

2

Photoallergen must bind to carrier protein

3

Develops after repeated exposures

4

Produces an eczematous reaction

5

Results in direct tissue injury Q/Q(M)-476784 Report a Problem

Which of the following is true of a phototoxic reaction? 5

Results in direct tissue injury

Phototoxic reactions result in direct tissue tiss ue injury when the phototoxic agent interacts with light energy to form reactive oxygen species. It is characterized by an eruption similar to a sunburn. Q/Q(M)-476784 Report a Problem This medicine causes degranulation of mast cells 39

1

NSAIDs

2

ASA

3

opiates

4

polymixin B

5

All of these answers are correct Q/Q(M)-474099 Report a Problem

This medicine causes degranulation of mast cells 5

All of these answers are correct

Mast cell degranulators include NSAIDs, ASA, opiates, polymixin B, radiocontrast, tubocurarine, succinylcholine, and others. Q/Q(M)-474099 Report a Problem Herpes gestationis is most commonly associated with: 1

Lymphoma

2

Multiparity

3

Grave's disease

4

Inflammatory bowel disease

5

Lupus Q/Q(M)-474189 Report a Problem

Herpes gestationis is most commonly associated with: 3

Grave's disease

Herpes gestationis (HG) typically occurs in the s econd or third trimester, and clinically presents a s urticarial papules and plaques around the umbilicus which progress to involve t he rest of the body. HG has been associated with Grave's disease. Q/Q(M)-474189 Report a Problem Direct immunofluorescent studies in chronic bullous disease of childhood is most likely to show: 1

Iga depostion in the superficial blood vessels

2

Linear IgG at the basement membrane

3

Linear IgA at the basement membrane

4

Granular IgG

5

Linear C3 at the basement membrane Q/Q(M)-474588 Report a Problem

Direct immunofluorescent studies in chronic bullous disease of childhood is most likely to show: 3

Linear IgA at the basement membrane

Chronic bullouse disease of childhood is believed to be a variant of linear IgA bullous disease, since disease are characterized characteri zed by antibodies to the 97kDa antigen that is a part of BPAg2. Children present with extremely pruritic blisters in i n circular arrangements. Pathology shows a subepidermal bullae with 40

neutrophils at the dermo-epidermal junction; the DIF shows IgA deposition at t he basement membrane in a linear fashionl. Q/Q(M)-474588 Report a Problem In staphylococcal scalded skin syndrome, the exfoliative toxin cleaves: 1

Desmoglein 1

2

Desmoglein 3

3

Desmocollin 1

4

Desmocollin 3

5

BpAg2 Q/Q(M)-474210 Report a Problem

In staphylococcal scalded skin syndrome, the exfoliative toxin cleaves: 1

Desmoglein 1

Staphylococcal scalded skin syndrome is caused by Staphylococci group 2, phage typ 71, which  produces an exofliative toxin that cleaves desmoglein 1. Clinically, this results in a subcorneal separation of keratinocytes, leading to skin tenderness, er ythema, and superficial desquamatioin of skin. Q/Q(M)-474210 Report a Problem Common cause(s) of drug-induced pemphigus foliaceus: 1

Captopril

2

Penicillamine

3

Captopril and penicillamine

4

Methotrexate

5

Dilantin Q/Q(M)-474632 Report a Problem

Common cause(s) of drug-induced pemphigus foliaceus: 3

Captopril and penicillamine

Penicillamine and captopril are common causes of drug-induced pemphigus. Q/Q(M)-474632 Report a Problem IgA pemphigus is a pustular acantholytic dermatosis with intracellular IgA deposition. The most common target on keratinocytes with patients with IgA pemphigus is: 1

Desmocollin1

2

Dsg2

3

IgG

4

C3

5

IgM 41

Q/Q(M)-482897 Report a Problem IgA pemphigus is a pustular acantholytic dermatosis with intracellular IgA deposition. The most common target on keratinocytes with patients with IgA pemphigus is: 1

Desmocollin1

IgA pemphigus is a pustular acantholytic dermatosis that targets desmocollin 1. Other targets can include Dsg1 and Dsg3. The pathogenesis of IgA pemphigus is unknown but can be associated with gammopathy. Q/Q(M)-482897 Report a Problem Latex allergy can cross-react with all except: 1

Avocado

2

Banana

3

Kiwi

4

Chestnut

5

Artichoke Q/Q(M)-474646 Report a Problem

Latex allergy can cross-react with all except: 5

Artichoke

Latex allergy can cross-react with wit h avocados, bananas, kiwis, chestnuts, and other foods. Artichokes are a member of the Asteraceae (formerly Compositae) family. Q/Q(M)-474646 Report a Problem What percentage of dermatitis herpetiformis patients are asymptomatic but have findings consistent with celiac sprue on gastrointestinal biopsy? 1

5%

2

15%

3

40%

4

70%

5

95% Q/Q(M)-474640 Report a Problem

What percentage of dermatitis herpetiformis patients are asymptomatic but have findings consistent with celiac sprue on gastrointestinal biopsy? 5

95%

Most patients with dermatitis herpetiformis have findings consistent with celiac disease on biopsy, but not all of them are symptomatic. Q/Q(M)-474640 Report a Problem The best first line treatment for dermatitis herpetiformis is: 1

Methotrexate 42

2

Dapsone

3

Colchicine

4

Prednisone

5

IVIG Q/Q(M)-474610 Report a Problem

The best first line treatment for dermatitis herpetiformis is: 2

Dapsone

The most effective drug for dermatitis herpetiformis is dapsonel. The dose varies between 50 and 300mg daily. Side effects include hemolytic anemia, leukopenia, methemoglobinemia, and rarely agranulocytosis or peripheral neuropathy. Sulfapyridine is also a very effective treatment for dermatitis herpetiformis. Q/Q(M)-474610 Report a Problem Antibodies to which antigen are most likely responsible for this disease? 1

Bullous pemphigoid antigen II

2

Desmoglein I

3

Desmocollin

4

Keratin 5

5

Plakoglobin Q/Q(M)-476509 Report a Problem

Antibodies to which antigen are most likely responsible for this disease? 1

Bullous pemphigoid antigen II

The clinical appearance of tense bullae and urticarial plaques is suggestive of bullous pemphigoid. Bullous pemphigoid antigen II is a transmembrane protein in the hemidesmosome. Antibodies to this antigen are pathogenic in bullous pemphigoid. Q/Q(M)-476509 Report a Problem The most important group of disease linked with the pathogenesis of paraneoplastic pemphigus in adults is 43

1

Lymphoproliferative disorders

2

Colerectal adenocarcinoma

3

Breast Cancer

4

Sarcomas

5

Melanoma Q/Q(M)-478692 Report a Problem

The most important group of disease linked with the pathogenesis of paraneoplastic pemphigus in adults is 1

Lymphoproliferative disorders

The most important group of disease linked witht eh pathogenesis of pareneoplastic pemphigus is Lymphoproliferative disorders. Most commonly non-Hodgkin's lymphoma, CLL, Castleman's tumor, sarcoma, and thymoma. Q/Q(M)-478692 Report a Problem Which of the following agent(s) has been most effective in treating severe ocular cicatricial  pemphigoid? 1

Cyclophosphamide

2

Mycophenolate mofetil

3

Cyclophosphamide + corticosteroids

4

Cyclosporin

5

Azathioprine Q/Q(M)-482144 Report a Problem

Which of the following agent(s) has been most effective in treating severe ocular cicatricial  pemphigoid? 3

Cyclophosphamide + corticosteroids

Cicatricial pemphigoid is a heterogeneous group of subepithelial blister ing diseases involving the mucous membranes and skin. The treatment of cictracial pemphigoid is predicated upon the extent, severity, and location of disease. Most regimens used ar e empiric and are based on clinical experience. e xperience. With severe ocular involvement, most experts recommend aggressive treatment with the combination of cyclophosphamide and steroids. Q/Q(M)-482144 Report a Problem Patients with epidermolysis bullosa acquisita have: 1

A defect in plectin

2

A defect in collagen XVII

3

Antibodies to collagen XVII

4

Antibodies to collagen VII

5

A defect in laminin 5 Q/Q(M)-474590 Report a Problem 44

Patients with epidermolysis bullosa acquisita have: 4

Antibodies to collagen VII

Epidermolysis bullosa acquisita (EBA) classically classicall y presents in adulthood as noninflammatory traumainduced bullae that heal with scarring, especially on the hands and feet. Histology shows a subepidermal  blister (usually pauci-inflammatory); DIF shows linear IgG at the basememt membrane; salt-split skin shows antibodies at the "floor". Patients hae antibodies to type VII collagen. Q/Q(M)-474590 Report a Problem This is derived from Myroxylon pereirae: 1

Thimerosol

2

Aniline dyes

3

Balsam of Peru

4

Colophony

5

Thiuram Q/Q(M)-474630 Report a Problem

This is derived from Myroxylon pereirae: 3

Balsam of Peru

Balsam of Peru is derived from the Myroxylon pereriae tree. Thimerosal is a questionable cause of allergic contact dermatitis; it is found in vaccines and contact lens solutions. Q/Q(M)-474630 Report a Problem Which of the following forms of epidermolysis bullosa is autosomal recessive? 1

Weber-Cockayne

2

Dowling-Meara

3

Junctional EB with pyloric atresia

4

EB simplex with muscular dystrophy

5

Hyperplastic cockayne-touraine Q/Q(M)-474604 Report a Problem

Which of the following forms of epidermolysis bullosa is autosomal recessive? 3

Junctional EB with pyloric atresia

The forms of junctional epidermolysis bullosa are inherited in a n autosomal recessive fashion. These include Herlitz, non-Herlitz, JEB with pyloric atresia, JEB inversa, and GABEB. Recessive dystrophic EB (Hallopeau-Siemens) is also a recessive subtype of dystrophic EB. EB simplex is typically inherited in an autosomal dominant fashion. Hyperplastic cockayne-touraine is a subtype of dominant dystrophic EB. Q/Q(M)-474604 Report a Problem Etyhlenediamine dihydrochloride is known to cross-react with all the following substances except? 1

Neomycin 45

2

Aminophylline

3

Promethazine

4

Meclizine

5

Hydroxyzine Q/Q(M)-482261 Report a Problem

Etyhlenediamine dihydrochloride is known to cross-react with all the following substances except? 1

Neomycin

Ethylenediamine dihydrochloride is a stabilizer found in aminophylline, theophylline, antihistamine creams, and Mycolog cream (nystatin/triamcinolone). It i s also used in synthetic coolants, rubber accelerators, insecticides, and fungicides. It is not known to cross react with neomycin. Q/Q(M)-482261 Report a Problem Formaldehyde releasers include all except: 1

Methylchoroisothiazolinone

2

Germall

3

Quaternium � 15

4

Dowicil

5

DMDM hydantoin Q/Q(M)-474647 Report a Problem

Formaldehyde releasers include all except: 1

Methylchoroisothiazolinone

Methylchloroisothiazolinone is also known as Kathon CG and it is a preservative. Germall, quaternium 15 (dowicil 200), DMDM hydantoin, and others are formaldehyde-releasing preservatives. Q/Q(M)-474647 Report a Problem An elderly gentleman with a history of hypertension and a thymoma, presents to the Emergency Room with oral and conjunctival erosions and hemorrhagic bullae on his hands and feet. These finds are seen in: 1

Cicatricial pemphigoid

2

Sweet�s syndrome

3

Toxic epidermal necrolysis

4

Paraneoplastic pemphigus

5

Erythema multiforme Q/Q(M)-477333 Report a Problem

An elderly gentleman with a history of hypertension and a thymoma, presents to the Emergency Room with oral and conjunctival erosions and hemorrhagic bullae on his hands and feet. These finds are seen in: 4

Paraneoplastic pemphigus 46

Paraneoplastic pemphigus is usually associated with cancers of lymphoid origin, including lymphoma, leukemia, thymomas, Castleman�s tumor. Clinically, this condition may present with intractable staomatitis, severe painful oral and conjunctival erosions. Q/Q(M)-477333 Report a Problem Which of the following is known as "prickly heat"? 1

Miliaria crystallina

2

Miliaria rubra

3

Miliaria profunda

4

Grover's disease

5

Fox-Fordyce disease Q/Q(M)-479630 Report a Problem

Which of the following is known as "prickly heat"? 2

Miliaria rubra

Miliaria are caused by occusion of ecrine sweat glands. Miliaria crystalina are clear minute subcorneal vesicles with no inflammation. Miliaria rubra are red papules subepidermal vesicles with wit h inflammation. Miliaria rubra is known as "prickly heat" as it presents after sweating, heat exposure, fever or occlusion. Miliaria profunda or pustulosa are red nodules or pustules with deeper inflammation. Fox-Fordyce disease is apocrine miliaria miliar ia that is very pruritic and presents in i n axilla and anogenital area of women. Grover's disease is a papulovesicular eruption of the trunk that is pruritic and can occur after sun exposure or in bedridden patients with occluded backs. Histologically Grover's disease presents with small foci of acantholytic dyskeratotic cells in the suprabasal region. Q/Q(M)-479630 Report a Problem What is the antigen found in the variant of cicatrical pemphigoid that is associated with internal malignancies? 1

BPAg1

2

BPAg2

3

Type VII collagen

4

Epiligrin

5

Keratins 5 & 14 Q/Q(M)-478659 Report a Problem

What is the antigen found in the variant of cicatrical pemphigoid that is associated with internal malignancies? 4

Epiligrin

Individuals with anti-epiligrin cicatricial pemphigoid are thought to be at an increased risk for solid internal malignancies. Q/Q(M)-478659 Report a Problem  Nickel can be detected in jewelry by applying: 47

1

Dimethylglyoxime

2

Methylchloroisothiazolinone

3

Dowicil

4

Quinone

5

Thiocyanate Q/Q(M)-474656 Report a Problem

 Nickel can be detected in jewelry by applying: 1

Dimethylglyoxime

Dimethylglyoxime application to metals containing nickel will show a pink color. Methylchloroisothiazolinone is Kathon CG, a preservative. Dowicil is a formaldehyde-releasing  preservative. Primin is a type of quinone, and it is the allergen in primrose (Primula obconica). Thiocyanates are the irritating substances in garlic. Q/Q(M)-474656 Report a Problem Perioral exuberant granulation tissue is sometimes s een in: 1

Cicatricial pemphigoid

2

Herlitz

3

Duhring's

4

Weber-Cockayne

5

Stevens-Johnson

Q/Q(M)-474628 Report a Problem Perioral exuberant granulation tissue is sometimes s een in: 2

Herlitz

The Herlitz form of junctional EB sometimes presents with excessive granulation tissue periorally, in the axillary/neck area. Q/Q(M)-474628 Report a Problem Which type of epidermolysis bullosa has the greatest risk of developing squamous cell carcinomas? 1

Weber-Cockayne

2

Dowling-Meara

3

Herlitz

4

EB Simplex with muscular dystrophy

5

Hallopeau-Siemens type of recessive dystrophic EB Q/Q(M)-474589 Report a Problem

Which type of epidermolysis bullosa has the greatest risk of developing squamous cell carcinomas? 5

Hallopeau-Siemens type of recessive dystrophic EB 48

Dystrophic EB is due to a defectin collagen 7, leading to decreas ed or absent anchoring fibrils in the sublamina densa. The three types of dominant dystrophic EB include Hyperplastic cockayne-Touraine, Albopapuloid Pasini, and Bart's. The Recessive form of dystrophic EB is also known as Hallpeau Siemens and patients present at birth owith generalized bullae which lead to erosions and atrophic scarring, mitten deformities, flexion contractures, oral and esophageal scarring and strictures and dysplastic teeth. There is an increased risk of squamous cell carcinomas. Q/Q(M)-474589 Report a Problem Herpes gestationis is exacerbated by: 1

Oral contraceptives

2

Menstruation

3

Third trimester

4

Postpartum state

5

All of these answers are correct Q/Q(M)-474634 Report a Problem

Herpes gestationis is exacerbated by: 5

All of these answers are correct

Herpes gestationis has also been associated with choriocarcinoma in several case reports. Q/Q(M)-474634 Report a Problem Which of the following immunosuppressive agents has been most effective in cicatricial pemphigoid? 1

Cyclophosphamide

2

Azathioprine

3

Mycophenolate mofetil

4

Cyclosporine

5

Methotrexate Q/Q(M)-477347 Report a Problem

Which of the following immunosuppressive agents has been most effective in cicatricial pemphigoid? 1

Cyclophosphamide

Cicatricial pemphigoid is a heterogeneous group of subepithelial blister ing diseases involving the mucous membranes and skin. The treatment of cictracial pemphigoid is predicated upon the extent, severity, and location of disease. Most regimens used ar e empiric and are based on clinical experience. e xperience. With severe ocular involvement, most experts recommend aggressive treatment with the combination of cyclophosphamide and steroids. Q/Q(M)-477347 Report a Problem A patients with a history of leukemia that had severe, s evere, persistent painful stomatitis extending from the lips into the pharynx, larynx and esophagus. Conjunctival involvement may lead to blindness. The cutaneous changes are polymorphic, ranging from erythematous macules to lichenoid papules to blisters and erosions. This patient most likely has:

49

1

Paraneoplastic pemphigus

2

Erythema multiforme

3

Cicatricial pemphigoid

4

Chemotherapy induced stomatitis

5

Persistent herpes simplex Q/Q(M)-482898 Report a Problem

A patients with a history of leukemia that had severe, s evere, persistent painful stomatitis extending from the lips into the pharynx, larynx and esophagus. Conjunctival involvement may lead to blindness. The cutaneous changes are polymorphic, ranging from erythematous macules to lichenoid papules to blisters and erosions. This patient most likely has: 1

Paraneoplastic pemphigus

This patient has paraneoplastic pemphigus. It is a n uncommon blistering disease with a variety of clinical patterns and target antigens associated with underlying malignancy. It is most likely associated with lymphoma, leukemia, Castleman tumor, or thymoma. Diagnosis is by indirect immunofluorescence is positive on rat bladder epithelium. Q/Q(M)-482898 Report a Problem Antibodies in chronic disease of childhood disease, map to the: 1

Basal layer

2

Lamina lucida

3

Lamina densa

4

Sublamina densa

5

Lamina lucida and sublamina densa

Q/Q(M)-474075 Report a Problem Antibodies in chronic disease of childhood disease, map to the: 5

Lamina lucida and sublamina densa

Immunoelectron mapping of chronic disease of childhood has shown binding within the lamina lucida as well as in the sublamina densa. This correlates corr elates with the finding of the 97-kDa antigen that i s homologous to a portion of the BPAg2 molecule as well as a 280-290 kDa antigen in this disease. Some have suggested that the 280-290 kDa antigen is type VII collagen, but the former does not co-migrate with type VII collagen on Western blots. Q/Q(M)-474075 Report a Problem Dermatitis herpetiformis is associated with: 1

HLA-Cw6

2

HLA-B27

3

HLA-DR4

4

HLA-DQ1

5

HLA-DR3 50

Q/Q(M)-474642 Report a Problem Dermatitis herpetiformis is associated with: 5

HLA-DR3

HLA DR3 and DQ2 are associated with dermatitis herpetiformis. H LA B27 is associated with psoriasis with spondylarthropathy, while HLA Cw6 is associated with psoriasis. Q/Q(M)-474642 Report a Problem Which steriod screening agent should be used when an allergic contact dermaititis is suspected to hydrocortisone? 1

Glutaral

2

Tixocortol-21-pivalate

3

Budesonide

4

Hydrocortisone-17-butyrate

5

Benzalkonium chloride Q/Q(M)-476780 Report a Problem

Which steriod screening agent should be used when an allergic contact dermaititis is suspected to hydrocortisone? 2

Tixocortol-21-pivalate

Tixocortol-21-pivalate is a group A steroid and screens for allergies to hydrocortisone, prednisone, and methylprednisolone. Budesonide screens for groups B and D steroids, and hydrocortisone-17-butyrate screens for group D steroids. Q/Q(M)-476780 Report a Problem Fecal isocoproporphyrin is seen in: 1

Porphyria cutanea tarda

2

Harderoporphyria

3

Variegate porphyria

4

Acute intermittent porphyria

5

Coproporphyria Q/Q(M)-473655 Report a Problem

Fecal isocoproporphyrin is seen in: 1

Porphyria cutanea tarda

Fecal isocoproporhyrin is characteristic of porphyria cutanea tarda. Q/Q(M)-473655 Report a Problem The Absoe-Hansen sign as applies to blister format ion refers to 1

Lateral slipping of the epidermis when perilesional is rubbed

2

Lateral dissection of the blister when pressure is applied directly to a blister 51

3

Blister formation following stroking of the lesion

4

Blister induction with incised trauma to skin

5

Blister formation after cryosurgery Q/Q(M)-478689 Report a Problem

The Absoe-Hansen sign as applies to blister format ion refers to 2

Lateral dissection of the blister when pressure is applied directly to a blister

The Asboe-Hansen sign as applies to blister formation refers to lateral dissection of the blister when  pressure is applied directly to the blister. This is also called, "bulla-spread phenomenon", because  pressure on an inact bulla, genlty forces fluid to wander under under the skin away from the pressure site. Q/Q(M)-478689 Report a Problem What is the most common cause of this entity? 1

Drugs

2

Infections

3

Malignancies

4

Idiopathic

5

UV exposure Q/Q(M)-482067 Report a Problem

What is the most common cause of this entity? 1

Drugs

Stevens-Johnson syndrome (SJS) is an immune-complex-“mediated immune-complex- “mediated hypersensitivity complex that is a severe expression of erythema multiforme. Most authors a uthors and experts consider SJS and toxic epidermal necrolysis (TEN) different manifestations of the same disease. The most common cause of recurrent episodes are erythema multiforme (AKA herpes associated as sociated erythema multiforme (HAEM)), whereas drugs are the most common cause of SJS/TEN. Q/Q(M)-482067 Report a Problem An older patient with erythema and crusting in a butterfly distribution and seborrheic areas with erosions is diagnosed with pemphigus erythematosus. The other synonym for pemphigus erythematosus is: 1

Senear-Usher syndrome

2

Wildfire pemphigus 52

3

Fogo Selvagem

4

Acrokeratosis verruciformis Hopf

5

Foliaceus erythematosus

Q/Q(M)-482896 Report a Problem An older patient with erythema and crusting in a butterfly distribution and seborrheic areas with erosions is diagnosed with pemphigus erythematosus. The other synonym for pemphigus erythematosus is: 1

Senear-Usher syndrome

Pemphigus erythematosus is also known as Senear-Usher syndrome. It is an uncommon variant of  pemphigus foliaceus with additional features of lupus erythematosus. It is most likely triggered by sunlight or medications than other forms of pemphigus foliaceus. Q/Q(M)-482896 Report a Problem Which is incorrect? 1

Tulipalin : alstroemeria

2

Urushiol : anacardia

3

Sesquiterpene lactones : artichokes

4

Usnic acid : lichen

5

Diallyl disulfide : fig Q/Q(M)-474653 Report a Problem

Which is incorrect? 5

Diallyl disulfide : fig

Tulipalin A is a byproduct of tuliposide A metabolism, and it is the allergen in alstroemeria, the Peruvian lily, and tulips. Urushiol is the allergen in Toxicodendron plants as well as several other cross reactors including cashew nut shells (Anacardium occidentale). Artichokes are in the family Asteraceae (formerly Compositae); this family is allergenic secondary to sesquiterpene lactones. Lichens contain usnic acid. The fig tree is in the family Moraceae, which is one of the plant families that can cause  phytophotodermatitis. Q/Q(M)-474653 Report a Problem Atopic dermatitis is associated with all except: 1

Lchthyosis hystrix

2

Central facial pallor

3

Pityriasis alba

4

Nipple eczema

5

Hyperlinear palms Q/Q(M)-474625 Report a Problem

Atopic dermatitis is associated with all except:

53

1

Lchthyosis hystrix

Icthyosis vulgaris (not hystrix) is associated with atopic dermatitis as one of the minor criteria of Hanifin. Q/Q(M)-474625 Report a Problem

This disease is inherited in an X-linked recessive manner: 1

Chronic granulomatous disorder

2

Job�s syndrome

3

Dowling-Meara

4

Mongomery�s syndrome

5

Treacher-Collins Q/Q(M)-474662 Report a Problem

This disease is inherited in an X-linked recessive manner: 1

Chronic granulomatous disorder

Chronic granulomatous disorder is inherited in an X-linked recessive manner, as is Wiskott-Aldrich. Job�s syndrome is autosomal dominant. Dowling-Meara is a subtype of EB simplex, with autosomal dominant inheritance. Mongomery�s syndrome is xanthoma disseminatum. Treacher Collins syndrome is also known as mandibulo-facial-dysostosis. Q/Q(M)-474662 Report a Problem A child develops an allergic reaction at the site of an influenza vaccine. To which of the following substances may she be allergic to? 1

Lanolin

2

Thimerosol

3

Ethylenediamine dichloride

4

Triclosan

5

Gluteraldehyde Q/Q(M)-482379 Report a Problem

A child develops an allergic reaction at the site of an influenza vaccine. To which of the following substances may she be allergic to? 2

Thimerosol

Thimerosol is a preservatives in vaccines such as the influenza, tetanus, and diphtheria vaccines. It is also found in antitoxins and immunoglobulins. Thimerosol is a mercury-containing organic compound. Lanolin is from the sebum of sheep. Ethylenediamine dichloride is a stabilizer in topical creams, medicines, dyes, insecticides, and fungicides and was previousl y found in nystatin cream. Triclosan is an antibacterial agent found in soap, shampoo and mouthwash. Gluteraldehyde is a cold sterilizing solution used for medical and dental equipment. Q/Q(M)-482379 Report a Problem

54

Antibodies against type VII collagen are seen in: 1

Epidermolysis bullosa simplex

2

Pemphigus erythematosus

3

Cicatricial pemphigoid

4

Epidermolysis bullosa acquisita

5

Bullous pemphigoid Q/Q(M)-477155 Report a Problem

Antibodies against type VII collagen are seen in: 4

Epidermolysis bullosa acquisita

Type VII collagen is present in the basement membrane of stratified squamous epithelia in the anchoring fibrils. It is the target tar get antigen in several blistering disease including i ncluding epidermolysis bullosa acquista,  bullous lupus erythematosus, dominant and recessive dystrophic epidermolysis bullosa. Q/Q(M)-477155 Report a Problem The agent most likely to cause pseudoporphyria is: 1

NSAID's

2

Penicillin

3

Beta-blockers

4

Vancomycin

5

Ace-inhibitors Q/Q(M)-474594 Report a Problem

The agent most likely to cause pseudoporphyria is: 1

NSAID's

 NSAIDS,especially naprosyn, are the most common group group of drugs implicated in pseudoporphyria. pseudoporphyria. Other common offenders include lasix, tetracycline, accutane, amiodarone and tanning beds. Q/Q(M)-474594 Report a Problem Which of the following proteins is typically NOT found to be antigenic in paraneoplastic pemphigus? 1

BPAg1

2

BPAg2

3

Desmoplakin

4

Envoplakin

5

Periplakin Q/Q(M)-474606 Report a Problem

Which of the following proteins is typically NOT found to be antigenic in paraneoplastic pemphigus? 2

BPAg2 55

BPAg2, a 180kDa protein, is typically t ypically not found to be antigenic in i n paraneoplastic pemphigus. The  proteins typically involved are: desmplakin 1 (250kDa) (250kDa) BPAg1 (230 kDa) Envoplakin Envoplakin (210kDa) Desmoplakin 2(210kDa) Periplakin (190 kDa) Unknown protein (170 kDa) Desmogleins 1& 3 (160 and 130 kDa) Q/Q(M)-474606 Report a Problem Which of the following chemicals is the most common cause of shoe contact dermatitis? 1

Thiuram mix

2

2-Mercaptobenzothiazole

3

Carba mix

4

Mixed dialkyl thioureas

5

Formaldehyde Q/Q(M)-474137 Report a Problem

Which of the following chemicals is the most common cause of shoe contact dermatitis? 2

2-Mercaptobenzothiazole

2-Mercaptobenzothiazole is found in the soles of shoes, and is the #1 cause of shoe contact dermatitis. It is also found in rubber accelerators, adhesives/tape, coolants, rubber, flea/tick powder, and support hose. Thiuram mix, carba mix, and mixed dialkyl thioureas are also rubber sensitizers. Thiuram is the second most frequent source of shoe contact dermatitis. dermatitis . Formaldehyde is a preservative and not found in shoes. Q/Q(M)-474137 Report a Problem The croton plant is irritating secondary to: 1

Calcium oxalate

2

Thiocyanate

3

Protoanemonin

4

Phorbol esters

5

Capsaicin Q/Q(M)-474631 Report a Problem

The croton plant is irritating secondary to: 4

Phorbol esters

Irritant contact dermatitis secondary s econdary to the croton plant is due to phorbol esters. Calcium oxalate is also an irritant; it is found in the "dumb caine" plant, a common house plant (Dieffenbachia) as well as in daffodils. Thiocyanates are irritants found in garlic. Protoanemonins are irritants found in buttercups. Q/Q(M)-474631 Report a Problem Which pair is not associated with allergic cross-reactivity? 1

Thimerosal : piroxicam

2

Ethylenediamine : hydroxyzine

3

PABA : procaine 56

4

Balsam of Peru : cinnamon

5

Thiuram : sulfa drugs Q/Q(M)-474644 Report a Problem

Which pair is not associated with allergic cross-reactivity? 5

Thiuram : sulfa drugs

Thiurams may cross-react with Antabuse (disulfiram). Q/Q(M)-474644 Report a Problem A woman in her 2nd trimester of pregnancy presents to clinic with urticarial plaques and papules around her umbilicus, chest and extremities. Tense vesicles are present within a few fe w of the erythematous  plaques. This womanâ™s condition is most commonly associated with : 1

Lymphoma

2

Multiparity

3

Graveâ™s Graveâ™s Disease

4

Inflammatory Bowel Disease

5

Lupus Q/Q(M)-482143 Report a Problem

A woman in her 2nd trimester of pregnancy presents to clinic with urticarial plaques and papules around her umbilicus, chest and extremities. Tense vesicles are present within a few fe w of the erythematous  plaques. This womanâ™s condition is most commonly associated with : 3

Graveâ™s Disease Graveâ™s

Herpes gestationis (HG) typically occurs in the s econd or third trimester, and clinically presents a s urticarial papules and plaques around the umbilicus which progress to involve the res t of the body. HG has been associated with Grave's disease. Hormonal factors influence the disease manifestation. This condition can be seen in pregnant women, menstruating women, and women taking oral contraceptives. Q/Q(M)-482143 Report a Problem Transient bullous disorder of childhood has a defect in: 1

Alpha6-integrin

2

Plectin

3

BPAg2

4

Epiligrin 57

5

Collagen VII Q/Q(M)-474652 Report a Problem

Transient bullous disorder of childhood has a defect in: 5

Collagen VII

Transient bullous dermolysis of the newborn may be a forme fruste/variant of dominant dystrophic EB. Mutations have been found in type VII collagen. Q/Q(M)-474652 Report a Problem Chloracne may be secondary to exposure to: 1

Chloroacetophenone

2

Hydrochloric acid

3

Hydrocarbons

4

Dioxin

5

Methylchloroisothiazolinone Q/Q(M)-474663 Report a Problem

Chloracne may be secondary to exposure to: 4

Dioxin

Chlorinated compounds can cause irritant dermatitis and sometimes chloracne. Chlorinated compounds include chloronaphthalene, chlorodiphenyl, dichlorobenzonitrile, tetrachloroazooxybenzene; dioxin (exposure in the Vietnam war); and cutting oils. Chloroacetophenone is an irrit ant in tear gas. Q/Q(M)-474663 Report a Problem

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