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Clinical and Developmental Immunology
Volume 2012, Article ID 369546, 9 pages
Review Article

Clinical Relevance of Autoantibodies in Patients with Autoimmune Bullous Dermatosis

Department of Dermatology and Allergology, Albert Szent-Györgyi Medical Center, University of Szeged, Korányi Fasor 6, 6720 Szeged, Hungary

Received 4 November 2012; Accepted 10 December 2012

Academic Editor: Dimitrios P. Bogdanos

Copyright © 2012 Lilla Mihályi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the “ladder” configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.