Pemphigus and Bullous Pemphigoid

Abstract

Pemphigus disorders are characterized by acantholysis, whereas pemphigoid disorders are characterized by a dermal-epidermal split. Diagnosis of pemphigus or pemphigoid relies on a combination of positive anti-desmoglein or anti-collagen XVII serology, confirmatory direct immunofluorescence, and clinical features. Treatment for immunobullous disease revolves around various immunosuppressants, most often some combination of rituximab, prednisone, and IVIg. Paraneoplastic pemphigus is characterized by hemorrhagic crusting of the lips with positive indirect immunofluorescence on rat bladder epithelium, which should prompt a search for malignancy. Hailey-Hailey disease is a genetically mediated pemphigus that typically occurs in skin folds and responds to a number of agents including botulinum toxin, topical steroids, and other anecdotal therapies. This review contains 17 figures, 2 tables, and 109 references. Keywords: blister, pemphigus, bullous, rituximab, bullae, prednisone