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Blistering Disease Lecture Review Qs Flashcards

1
Q

what can cause/flare Grover disease

A

heat or bedrest

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2
Q

antibody in linear IgA disease

A

97-120 kD epitope of BPAg 2

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3
Q

drug commonly known to cause linear IgA ds

A

vancomycin

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4
Q

common antibodies in DH

A

anti-endomysial, anti-epidermal transglutaminase, anti-gliadin

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5
Q

appearance of DIF in DH

A

granular IgA along DEJ

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6
Q

diseases associated with DH

A

thyroid ds (38-50%)
sm bowel T-cell lymphoma
diabetes, addison’s ds, hepatitis, alopecia areata
sarcoidosis, scleroderma, Sjogren’s, SLE, vitiligo

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7
Q

presentation and HLAs of DH

A

pruritic vesicles on extensor elbows/knees, scalp and nuchal areas
HLA DQ2 & 8

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8
Q

another name for dermatitis herpetiformis

A

Duhring disease

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9
Q

antibody in epidermolysis bullosa acquisita

A

type 7 collagen (NC1 domain)

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10
Q

DOC for cicatricial pemphigoid with severe eye involvement. ineffective drug?

A 
DOC = cyclophosphamide
ineffective = cyclosporine
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11
Q

antibody in malignancy-associated cicatricial pemphigoid

A

laminin 332

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12
Q

antibody in ocular cicatricial pemphigoid

A

beta-4 integrin

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13
Q

antibody in cicatricial pemphigoid with mucosal and skin lesions

A

BPAg 2 (distal C terminal)

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14
Q

name for cicatricial pemphigoid on head and neck without oral involvement

A

Brunsting-Perry

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15
Q

skin lesions are seen in what % cicatricial pemphigoid

A

25%

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16
Q

cicatricial pemphigoid involves the conjunctiva and oral mucosa in what %

A

conjunctiva = 66%

oral mucosa = 90%

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17
Q

women with cholestasis of pregnancy are at risk for what postpartum and why

A

postpartum hemorrhage due to low vitamin k (fetus also at risk)

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18
Q

lab abnormality in impetigo herpetiformis

A

hypocalcemia (hypoparathyroidism)

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19
Q

is there risk to mother and fetus in PUPPP

A

no

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20
Q

areas spared in PUPPP

A

periumbilical, upper chest, face, mucous membranes, palms and soles

21
Q

ds associated with pemphigoid gestationis and HLA associated

A

grave’s ds

HLA DR3 and DR4

22
Q

antibody in pemphigoid gestationis and domain

A

BPAg 2 NC16a domain

23
Q

trimester that pemphigoid gestationis occurs

24
Q

common drug known to cause BP

A

furosemide

25
Q

bullous pemphigoid antigens and domain. HLA assocaited

A

BPAg 2 NC16a domain and BPAg 2

HLA-DQB1

26
Q

IIF in BP

27
Q

DIF in BP

A

linear C3 and IgG4 at basement membrane

28
Q

% in BP with oral involvement

29
Q

process associated with poor prognosis in paraneoplastic pemphigus

A

bronchiolitis obliterans

30
Q

2 types of IgA pemphigus and antibodies

A

subcorneal pustular dermatosis (desmocollin 1)

intraepidermal neutrophilic dermatosis (desmoglein 1/3)

31
Q

most consistent symptom of paraneoplastic pemphigus

A

intractable stomatitis

32
Q

malignancy in adolescents with paraneoplastic pemphigus

A

Castleman’s disease

33
Q

malignancies associated with paraneoplastic pemphigus

A

NHL (40%), CLL (30%), Castleman’s ds (10%), Thymoma/Sarcoma/Waldenstrom’s (6% each)

34
Q

antibody in pemphigus erythematosus

A

anti-desmoglein 1

35
Q

another name for pemphigus erythematosus and what other ds does it share features with

A

Senear-Usher syndrome

lupus

36
Q

arthropod bite possibly related to fogo selvalgem

A

simulium nigrimanum

37
Q

country endemic pemphigus is found and age if affects

A

Brazil

children and young adults

38
Q

histology of pemphigus foliaceus

A

subcorneal/intragranular acantholysis with ‘cling ons’

39
Q

drugs implicated in pemphigus foliaceus

A

thiol drugs (captopril, gold thiosulfate), sulfa drugs, penacillamine, nifedipine

40
Q

does pemphigus foliaceus have oral lesions

41
Q

2 subtypes of pemphigus vegetans

A

neuman (MC and severe)

hallopeau (mild, pustular)

42
Q

DIF pattern in pemphigus vulgaris

A

chickenwire with IgG4

43
Q

histology in pemphigus vulgaris

A

suprabasilar acantholysis with tomb-stoning and eosinophilic spongiosus

44
Q

mucocutaneous variant of pemphigus vulgaris due to what antibodiy? and mucosal?

A

mucocutaneous: anti-desmoglein 1&3
mucosal: anti-desmoglein 3

45
Q

HLA associated with pemphigus vulgaris

A

HLA DR4 & DR6

46
Q

where do blisters commonly first appear in pemphigus vulgaris

47
Q

what is the sign when pressure on blister surface results in lateral spread?
when slight rubbing of skin reveals the dermis?

A

Asboe-Hansen sign

Nikolsky sign

48
Q

medium used for transport of tissue for DIF

A

Michel’s media

49
Q

tissue for IIF for pemphigus foliaceus? for paraneoplastic pemphigus? for pemphigus vulgaris and DH?

A

PF = guinea pig esophagus
PNP = rat bladder
PV and DH = monkey esophagus

Andrews (15 decks)

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