Pemphigus herpetiformis: From first description until now - 14/03/14

Doi : 10.1016/j.jaad.2013.11.043

Michael Kasperkiewicz, MD ^a,^⁎ , Cezary Kowalewski, MD, PhD ^b, Stefania Jabłońska, MD, PhD ^b
^a Department of Dermatology, University of Lübeck, Lübeck, Germany
^b Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland

^∗Correspondence to: Michael Kasperkiewicz, MD, Department of Dermatology; University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany.

Abstract

Pemphigus herpetiformis is one of the less common forms of pemphigus, first introduced by Jabłońska and colleagues in 1975. This autoimmune bullous disease combines the clinical features of dermatitis herpetiformis and the immunologic characteristics of pemphigus. The target autoantigen is usually desmoglein 1 (or less frequently desmoglein 3), although recently it has become increasingly obvious that patients with pemphigus herpetiformis also demonstrate autoantibodies against desmocollin. Here, we summarize reported cases of pemphigus herpetiformis and describe current knowledge considering epidemiology, clinical manifestations, histologic findings, immunopathology, pathophysiologic concepts, associated diseases, and treatment of this rare disorder.

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Key words : autoantibody, autoantigen, autoimmunity, bullous disease, desmoglein, pemphigus

Abbreviations used : Dsc, Dsg, PF, PH, PV