Blistering Disease Lecture Review Qs Flashcards

(49 cards)

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

25
bullous pemphigoid antigens and domain. HLA assocaited
BPAg 2 NC16a domain and BPAg 2 | HLA-DQB1
26
IIF in BP
roof
27
DIF in BP
linear C3 and IgG4 at basement membrane
28
% in BP with oral involvement
20%
29
process associated with poor prognosis in paraneoplastic pemphigus
bronchiolitis obliterans
30
2 types of IgA pemphigus and antibodies
subcorneal pustular dermatosis (desmocollin 1) | intraepidermal neutrophilic dermatosis (desmoglein 1/3)
31
most consistent symptom of paraneoplastic pemphigus
intractable stomatitis
32
malignancy in adolescents with paraneoplastic pemphigus
Castleman's disease
33
malignancies associated with paraneoplastic pemphigus
NHL (40%), CLL (30%), Castleman's ds (10%), Thymoma/Sarcoma/Waldenstrom's (6% each)
34
antibody in pemphigus erythematosus
anti-desmoglein 1
35
another name for pemphigus erythematosus and what other ds does it share features with
Senear-Usher syndrome | lupus
36
arthropod bite possibly related to fogo selvalgem
simulium nigrimanum
37
country endemic pemphigus is found and age if affects
Brazil | children and young adults
38
histology of pemphigus foliaceus
subcorneal/intragranular acantholysis with 'cling ons'
39
drugs implicated in pemphigus foliaceus
thiol drugs (captopril, gold thiosulfate), sulfa drugs, penacillamine, nifedipine
40
does pemphigus foliaceus have oral lesions
no
41
2 subtypes of pemphigus vegetans
neuman (MC and severe) | hallopeau (mild, pustular)
42
DIF pattern in pemphigus vulgaris
chickenwire with IgG4
43
histology in pemphigus vulgaris
suprabasilar acantholysis with tomb-stoning and eosinophilic spongiosus
44
mucocutaneous variant of pemphigus vulgaris due to what antibodiy? and mucosal?
mucocutaneous: anti-desmoglein 1&3 mucosal: anti-desmoglein 3
45
HLA associated with pemphigus vulgaris
HLA DR4 & DR6
46
where do blisters commonly first appear in pemphigus vulgaris
mouth
47
what is the sign when pressure on blister surface results in lateral spread? when slight rubbing of skin reveals the dermis?
Asboe-Hansen sign | Nikolsky sign
48
medium used for transport of tissue for DIF
Michel's media
49
tissue for IIF for pemphigus foliaceus? for paraneoplastic pemphigus? for pemphigus vulgaris and DH?
PF = guinea pig esophagus PNP = rat bladder PV and DH = monkey esophagus