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Giornale Italiano di Dermatologia e Venereologia 1999 August;134(4):321-9

Copyright © 1998 EDIZIONI MINERVA MEDICA

language: Italian

New clinical variants of pemphigus

Fabbri P., Caproni M., Brazzini B.

Università degli Studi - Firenze, Clinica Dermatologica II


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Recent immunopathologic research on Pemphigus permitted to include in this group of dermatosis a series of clinical variants, that had been considered atypical manifestations of other bullous diseases or associations with different dermatosis or variants that had not been yet classified. Three clinical forms, pemphigus herpetiformis, IgA-pemphigus and paraneoplastic pemphigus, nowadays definable as “new clinical variants of pemphigus” will be examined. Each variant has its own specific clinical expression, even if similar to other common diseases. Pemphigus herpetiformis is a chronic disease with frequent relapses, characterized by the eruption of intensely pruritic erythematous wheals, papules and vesicles; it also shows a good response to suefons. Circulating autoantibodies in pemphigus herpetiformis bind to intercellular epidermal antigens represented by desmoglein 1 and desmoglein 3. IgA Pemphigus is characterized by a broad spectrum of cutaneous lesions and histopathologic features. All these manifestations have in common an intercellular deposition of IgA in the epidermis associated to accantholysis. The different sites of the IgA deposits seem to be related to the various clinical and histological variants. With the term “paraneoplastic pemphigus”, Anhalt described a muco-cutaneous dermatosis characterized by polymorphous clinical manifestations, specific histopathologic features and circulating autoantibodies that specifically bind to epidermal antigens (170kDa-250 kDa). Most of the cases of paraneoplastic pemphigus were described in association with lymphoreticular tumors, but in some cases reported in the literature, no tumor had been diagnosed.

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