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Systematic Review ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Immunol. | doi: 10.3389/fimmu.2019.02466

Anti-p200 pemphigoid: A Review

  • 1Department of Dermatology, Tufts University School of Medicine, United States
  • 2Center for Blistering Diseases, United States
  • 3Department of Dermatology, Rambam Health Care Campus, Israel

The many clinical aspects of anti-p200 pemphigoid are not well characterized. We aimed to analyze and correlate known existing data on the epidemiological, clinical, histological, and immunological features of anti-p200 pemphigoid. We performed a review using Medline, Embase and Web of Science databases(1900-2018). Case reports and series of patients were included. Sixty-eight eligible studies comprising 113 anti-p200 pemphigoid patients were included in the qualitative analysis, with a mean age of onset of 65.5 years. All patients presented with bullae/vesicles, 54.3% had urticarial plaques. A similarity to bullous pemphigoid was reported in 66.1% of cases, but palmoplantar (51.4%), cephalic (40.3%) and mucosal (38.5%) involvement, besides frequent development of scars/milia (15.7%) were reported. Autoantibodies against recombinant laminin γ1 were detected in the sera of 73.1% of patients. Psoriasis was present in 28.3% of anti-p200 pemphigoid patients, particularly among Japanese patients (56.4%). The incidence of pustular psoriasis in this subgroup, was significantly greater than the normal population. In conclusion, the diagnosis of anti-p200 pemphigoid may be suspected when a subepidermal autoimmune blistering disease develops in a younger age group, with significant acral and cephalic distribution and mucosal involvement.

Keywords: Anti-p200, Mucous membrane pemphigoid, laminin γ1 pemphigoid, salt-split skin, Immunoblotting, ELISA, Bullous pemphigoid, BMZ autoantibodies, Mucosal disease, Psoriasis, Pustular psoriasis

Received: 08 Aug 2019; Accepted: 03 Oct 2019.

Copyright: © 2019 Ahmed and Kridin. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Mx. A. Razzaque Ahmed, Tufts University School of Medicine, Department of Dermatology, Boston, United States,