Pemphigus: PV, PF, PNP, IgA pemphigus Flashcards
autoantigen of mucosal PV
desmoglein 3
autoantigen of mucocutaneous PV
desmoglein 1 and 3
drugs that can cause PV
penicillamine captopril enalapril lisinopril piroxicam
HLA associations with PV
HLA-DR4, DRw6, DR14
level of split on histology of PV
suprabasilar (tombstoning)
DIF of PV
intercellular IgG4 > C3 in net like pattern
IIF of PV
monkey esophagus
autoantigen in foliaceous
desmoglein 1
endemic form of PF (brazil)
Fogo selvagem
localized PF variant with SLE overlap
pemphigus erythematosus
drugs that can cause PF
pencilliamine
nifedipine
ACEi
level of split on histology of PF
subcorneal (acantholysis), look for PMNs in blister cavity
IIF substrate of PF
guinea pig
cancers a/w paraneoplastic pemphigus
non-hodgkin's lymphoma >> CLL, Castleman's disease (benign) Waldenstrom macroglobulinemia sarcoma Benign thymoma
autoantigen of paraneoplastic pemphigus (PNP)
lots of them …
histology/split level of PNP
suprabasilar + vacuolar basal cell damage w/ lichenoid infiltrate, look for necrotic keratinocytes
IIF substrate + findings
rat bladder
intercellular IgG
DIF PNP
intercellular IgG + linear IgG/C3 on BMZ
complication of PNP
BOOP
autoantigen in IgA pemphigus
desmocollin 1 (+/- desmoglein 1/3)
level of split/histology of IgA pemphigus
intraepidermal (NO acantholysis)
DIF IgA pemphigus
intercellular IgA
2 subtypes of IgA pemphigus
- subcorneal pustular dermatosis (serpiginous vesicles/pustules)
2 Intraepidermal neutrophillic type (sunflower like arrangement of flaccid vesicles/pustules in intertriginous locations
