Pemphigus: Current Concepts

doi:10.1059/0003-4819-92-3-396

Pemphigus: Current Concepts

Los Angeles, California

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.

Article and Author Information

▸An edited transcription of an Interdepartmental Clinical Case Conference arranged by the Department of Medicine of the UCLA School of Medicine; Los Angeles, California.
▸Authors who wish to cite a section of this conference and specifically indicate its author can use this example for the form of reference:

GRAHAM J. Pathology, pp. 398-9. In: AHMED AR, moderator. Pemphigus: current concepts. Ann Intern Med. 1980;92:396-405.
▸Requests for reprints should be addressed to A. Razzaque Ahmed, M.D.; Division of Dermatology, Department of Medicine, UCLA School of Medicine; Los Angeles, CA 90024.
- Received December 15, 1979.
- Accepted December 31, 1979.