Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

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Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus*

The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. Th...

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[Paraneoplastic pemphigus].

UNLABELLED A RARE DISEASE: Paraneoplastic pemphigus is an rare autoimmune bullous skin disease recently recognized. About 50 cases have been reported since its first description in 1990. CLINICAL MANIFESTATIONS Clinical signs are polymorphous resembling the cutaneomucosal manifestations of pemphigus vulgar (skin and mucosa erosions, fragile interdermal bullae), pemphigoid (urticaria, distende...

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Pemphigus herpetiformis with progression to pemphigus foliaceus: a case report.

Pemphigus herpetiformis (PH) is an uncommon variant of pemphigus that accounts for an estimated 6% to 7.2% of all cases of this skin disorder. The term pemphigus herpetiformis was coined by Jablonska et al. in 1975 to describe a entity that was clinically similar to dermatitis herpetiformis, showed acantholysis on biopsy, and responded to sulfapyridine. The authors considered that PH was a vari...

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Pemphigus Vulgaris and Pemphigus Foliaceus

The word “pemphigus” is derived from the Greek term "pemphix” meaning bubble or blister. Pemphigus is a group of autoimmune diseases (see Table 4) characterized by intra-epithelial blistering, resulting in superficial vesicles or bullae that easily rupture, resulting in ulceration of mucosal and/or cutaneous sites. Although rare, pemphigus causes significant morbidity and potential mortality fo...

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Paraneoplastic Pemphigus: Recent Advances

These confirmatory reports have verified all the essential features of the disease that was first described. Mucosal ulceration in the form of intractable stomatitis is the most constant clinical feature of the disease. In no case to-date has this been absent. Cutaneous manifestations including blisters, lichenoid eruption or erythema multiforme like lesions are highly variable. These can chang...

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ژورنال

عنوان ژورنال: Anais Brasileiros de Dermatologia

سال: 2014

ISSN: 0365-0596

DOI: 10.1590/abd1806-4841.20142459