Pemphigus vegetans Neumann type with anti-desmoglein and anti-periplakin autoantibodies.
Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by vegetating lesions in the folds and mouth and by the presence of autoantibodies against desmoglein 3. We describe two Caucasian patients with pemphigus vegetans, one of them presented antibodies to desmoglein 3 and 1 and the other one to desmoglein 3. Both patients also had circulating antibodies against a 190 kDa protein co-migrating with periplakin. Anti-periplakin reactivity is usually detected in paraneoplastic pemphigus, while it has never been reported in pemphigus vegetans. Our observation enlarges the spectrum of autoantibodies which may be associated with pemphigus vegetans. However, the pathophysiological significance of anti-periplakin reactivity in this pemphigus variant remains to be determined.
Cozzani
Emanuele
E
Section of Dermatology, University of Genoa, Viale Benedetto XV, n 7, 16132, Genova, Italy.
Christana
Konstantina
K
Mastrogiacomo
Alessandro
A
Rampini
Paolo
P
Drosera
Massimo
M
Casu
Massimo
M
Murialdo
Giovanni
G
Parodi
Aurora
A
D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D051183:Desmoglein 1 / Q000276:immunology*; D051184:Desmoglein 3 / Q000276:immunology*; D005260:Female; D006801:Humans; D007159:Immunologic Tests; D008297:Male; D010392:Pemphigus / Q000276:immunology* / Q000473:pathology; D051179:Plakins / Q000276:immunology*; D012867:Skin / Q000473:pathology
D001323:Autoantibodies; D051183:Desmoglein 1; D051184:Desmoglein 3; C491674:PPL protein, human; D051179:Plakins