(Figure 3.25B) - Less severe and more superficial than PV
- Autoantigen : desmoglein 1
| | | | | | Dsg 1 also in striate PPK | | | | | |
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- Clinical : flaccid bullae which rupture easily; often only erythematous patches, erosions, and crusting remain
- Fogo selvagem (endemic form of PF): seen in rural Brazil and clinically identical to PF, related to the black fly (Simulium spp.)
- Pemphigus erythematosus (Senear-Usher syndrome): localized PF variant with lupus erythematosus overlap; involves seborrheic areas with erythema, erosions and crusting; ANA positive in 30%; DIF: intercellular and linear IgG at BMZ; treat with sun protection, oral/ topical corticosteroid
- Drug-induced: penicillamine, nifedipine, ACEI
- Histology: subcorneal acantholysis with acantholytic cells seen on blister roof (‘cling ons’), neutrophils can be seen in blister cavity (resembling impetigo)
- DIF: same as PV (more pronounced in upper layers)
- IIF: + in 80% cases, best substrate is guinea pig esophagus
- Treatment: similar to PV
| | Figure 3.25
A: DIF, pemphigus vulgaris
(Courtesy of Dr. Paul Getz)
B: Pemphigus foliaceous
(Courtesy of Dr. Paul Getz)
C: Paraneoplastic pemphigus |
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