The expanding spectrum of IgA pemphigus: A case report and review of the literature

S. Geller, A. Gat, T. Zeeli, A. Hafner, R. Eming, M. Hertl, E. Sprecher

Research output: Contribution to journalReview articlepeer-review

Abstract

Summary IgA pemphigus (IGAP) is a rare, distinct variant of pemphigus, currently classified, depending upon the histological features, immunofluorescence staining pattern and autoantibody profile, into two types: subcorneal pustular dermatosis (SPD) and intraepidermal neutrophilic IgA dermatosis. In a patient with a widespread blistering disease of the skin resembling SPD-type IGAP, we demonstrate the coexistence of IgA reactivity to both epidermal (desmocollins 2 and 3) and basement membrane-associated (BP180) proteins, suggesting the coexistence of atypical IGAP and linear IgA bullous dermatosis, respectively. This case, together with 20 previous reports of atypical IGAP, underscores the limitations of current classification schemes. Therefore, we suggest reclassifying these cases under the general term 'IGAP spectrum'. What's already known about this topic? IgA pemphigus (IGAP) is a rare variant of pemphigus. Pemphigus is traditionally subdivided into subcorneal pustular dermatosis and intraepidermal neutrophilic IgA dermatosis. What does this study add? A case report and a review of previous reports pointing to the need to revise the current classification of IGAP. The possibility of the existence of a spectrum of IGAP-like diseases.

Original languageEnglish
Pages (from-to)650-656
Number of pages7
JournalBritish Journal of Dermatology
Volume171
Issue number3
DOIs
StatePublished - 1 Sep 2014
Externally publishedYes

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