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European Journal of Dermatology

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Pemphigus vegetans Neumann type with anti-desmoglein and anti-periplakin autoantibodies Volume 17, issue 6, November-December 2007

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Authors
Section of Dermatology, University of Genoa, Viale Benedetto XV, n. 7, 16132 Genova Italy, Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell’Immacolata IRCCS, Rome, Italy, Section of Endocrinology, University of Genoa, Italy

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.