The new pemphigus variants

Pemphigus describes a group of autoimmune diseases characterized by blister s and erosions of the skin and mucous membranes, acantholysis by histology, and autoantibodies directed against epidermal cell surface components. Sin ce the early 1970s, the following new clinical variants of pemphigus have b een reported: pemphigus herpetiformis, IgA pemphigus, and paraneoplastic pe mphigus. In recent years, significant data have been obtained from laborato ry investigation on these rare and atypical variants, especially regarding their specific target antigens. We review these variants, their clinical pr esentations, histologic findings, immunopathology, target antigens, theorie s of pathogenesis, treatment modalities, and clinical courses.