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IgA Pemphigus

  • Barbara HorváthEmail author
  • Marcel F. Jonkman
Chapter

Abstract

IgA pemphigus (IGAP) is a rare distinct variant of pemphigus characterized by tissue-bound and circulating autoantibodies exclusively from the IgA class against desmosomal and non-desmosomal proteins of the epidermis. Based on the clinics, histology, direct immunofluorescence, and autoantibody profile, it is classified in two types: the subcorneal pustulosis dermatosis (SPD)-type and the intraepidermal neutrophilic IgA dermatosis (IEN)-type. The first-line therapy is dapsone.

Keywords

IgA pemphigus (IGAP) Immunoglobulin A (IgA) Desmosomes Pustular disease 

References

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For Further Reading

  1. Hashimoto T, Komai A, Futei Y, Nishikawa T, Amagai M. Detection of IgA autoantibodies to desmogleins by an enzyme-linked immunosorbent assay: the presence of new minor subtypes of IgA pemphigus. Arch Dermatol. 2001;137:735–8.PubMedGoogle Scholar
  2. Geller S, Gat AA, Zeeli T, Hafner A, Eming R, Hertl M, Sprecher E. The expanding spectrum of IgA pemphigus: a case report and a review of the literature. Br J Dermatol. 2014;171:650–6.CrossRefPubMedGoogle Scholar
  3. Ishii N, Ishida-Yamamoto A, Hashimoto T. Immunolocalization of target autoantigens in IgA pemphigus. Clin Exp Dermatol. 2004;29:62–6.CrossRefPubMedGoogle Scholar
  4. Moreno AC, Santi CG, Gabbi TV, Aoki V, Hashimoto T, Maruta CW. IgA pemphigus: case series with emphasis on therapeutic response. J Am Acad Dermatol. 2014;70:200–1.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Department of DermatologyCenter for Blistering Diseases, University Medical Center Groningen, University of GroningenGroningenthe Netherlands

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