Pemphigus: current concepts

Abstract

Pemphigus is an autoimmune intraepidermal bullous disease involving the skin and mucous membranes. There are four clinically recognized variants of this disease. The histopathologic hallmark is acantholysis, which is disruption of normal cell-to-cell adhesion. Patients produce an IgG antibody directed against an antigen present in the intercellular substance of the epidermis. The antibody binds to the intercellular spaces in vivo and can be frequently detected in patients' sera. Immunofluorescent examination of perilesional tissue and serum is an extremely valuable diagnostic technique. Recently, pemphigus-like lesions have been produced in skin explants grown in tissue culture media enriched with pemphigus serum. Before corticosteroid therapy was available, pemphigus was a fatal disease. Steroids, and more recently immunosuppressive agents, have drastically improved the prognosis.