Pemphigus describes a group of autoimmune diseases characterized by blisters and erosions of the skin and mucous membranes, acantholysis by histology, and autoantibodies directed against epidermal cell surface components. Since the early 1970s, the following new clinical variants of pemphigus have been reported: pemphigus herpetiformis, IgA pemphigus, and paraneoplastic pemphigus. In recent years, significant data have been obtained from laboratory investigation on these rare and atypical variants, especially regarding their specific target antigens. We review these variants, their clinical presentations, histologic findings, immunopathology, target antigens, theories of pathogenesis, treatment modalities, and clinical courses. (J Am Acad Dermatol 1999;40:649-71.) Learning Objective: After completing this article, readers should be familiar with pemphigus herpetiformis, IgA pemphigus, and paraneoplastic pemphigus. On the basis of their unique clinical, histologic, and immunofluorescence findings, readers should also be able to differentiate these new pemphigus variants from the common forms of pemphigus.