Pemphigus herpetiformis: From first description until now

Michael Kasperkiewicz*, Cezary Kowalewski, Stefania Jabłońska

*Corresponding author for this work
19 Citations (Scopus)


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.

Original languageEnglish
JournalJournal of the American Academy of Dermatology
Issue number4
Pages (from-to)780-787
Number of pages8
Publication statusPublished - 01.01.2014


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