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

Abstract
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'.
AuthorsS Geller, A Gat, T Zeeli, A Hafner, R Eming, M Hertl, E Sprecher
JournalThe British journal of dermatology (Br J Dermatol) Vol. 171 Issue 3 Pg. 650-6 (Sep 2014) ISSN: 1365-2133 [Electronic] England
PMID24601812 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review)
Copyright© 2014 British Association of Dermatologists.
Chemical References
  • Autoantigens
  • DSC2 protein, human
  • DSC3 protein, human
  • Desmocollins
  • Immunoglobulin A
  • Non-Fibrillar Collagens
  • collagen type XVII
Topics
  • Adult
  • Aged
  • Aged, 80 and over
  • Autoantigens (immunology)
  • Child
  • Desmocollins (immunology)
  • Female
  • Humans
  • Immunoglobulin A (immunology)
  • Male
  • Middle Aged
  • Non-Fibrillar Collagens (immunology)
  • Pemphigus (immunology, pathology)

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