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Vesiculobullous diseases > Pemphigus: PV, PF, PNP, IgA pemphigus > Flashcards

Pemphigus: PV, PF, PNP, IgA pemphigus Flashcards

1
Q

autoantigen of mucosal PV

A

desmoglein 3

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2
Q

autoantigen of mucocutaneous PV

A

desmoglein 1 and 3

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3
Q

drugs that can cause PV

A 
penicillamine
captopril
enalapril 
lisinopril 
piroxicam
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4
Q

HLA associations with PV

A

HLA-DR4, DRw6, DR14

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5
Q

level of split on histology of PV

A

suprabasilar (tombstoning)

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6
Q

DIF of PV

A

intercellular IgG4 > C3 in net like pattern

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7
Q

IIF of PV

A

monkey esophagus

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8
Q

autoantigen in foliaceous

A

desmoglein 1

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9
Q

endemic form of PF (brazil)

A

Fogo selvagem

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10
Q

localized PF variant with SLE overlap

A

pemphigus erythematosus

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11
Q

drugs that can cause PF

A

pencilliamine
nifedipine
ACEi

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12
Q

level of split on histology of PF

A

subcorneal (acantholysis), look for PMNs in blister cavity

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13
Q

IIF substrate of PF

A

guinea pig

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14
Q

cancers a/w paraneoplastic pemphigus

A 
non-hodgkin's lymphoma >> CLL,
Castleman's disease (benign)
Waldenstrom macroglobulinemia 
sarcoma
Benign thymoma
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15
Q

autoantigen of paraneoplastic pemphigus (PNP)

A

lots of them …

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16
Q

histology/split level of PNP

A

suprabasilar + vacuolar basal cell damage w/ lichenoid infiltrate, look for necrotic keratinocytes

17
Q

IIF substrate + findings

A

rat bladder

intercellular IgG

18
Q

DIF PNP

A

intercellular IgG + linear IgG/C3 on BMZ

19
Q

complication of PNP

A

BOOP

20
Q

autoantigen in IgA pemphigus

A

desmocollin 1 (+/- desmoglein 1/3)

21
Q

level of split/histology of IgA pemphigus

A

intraepidermal (NO acantholysis)

22
Q

DIF IgA pemphigus

A

intercellular IgA

23
Q

2 subtypes of IgA pemphigus

A
  1. subcorneal pustular dermatosis (serpiginous vesicles/pustules)
    2 Intraepidermal neutrophillic type (sunflower like arrangement of flaccid vesicles/pustules in intertriginous locations

Vesiculobullous diseases (6 decks)

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