Epidermal transglutaminase (TGase 3) is the autoantigen of dermatitis herpetiformis

Miklós Sárdy, Sarolta Kárpáti, Barbara Merkl, Mats Paulsson, Neil Smyth

Research output: Contribution to journalArticle

377 Citations (Scopus)

Abstract

Gluten sensitivity typically presents as celiac disease, a common chronic small intestinal disorder. However, in certain individuals it is associated with dermatitis herpetiformis, a blistering skin disease characterized by granular IgA deposits in the papillary dermis. While tissue transglutaminase has been implicated as the major autoantigen of gluten sensitive disease, there has been no explanation as to why this condition appears in two distinct forms. Here we show that while sera from patients with either form of gluten sensitive disease react both with tissue transglutaminase and the related enzyme epidermal type 3 transglutaminase, antibodies in patients having dermatitis herpetiformis show a markedly higher avidity for epidermal transglutaminase. Further, these patients have an antibody population specific for this enzyme. We also show that the IgA precipitates in the papillary dermis of patients with dermatitis herpetiformis, the defining signs of the disease, contain epidermal transglutaminase, but not tissue transglutaminase or keratinocyte transglutaminase. These findings demonstrate that epidermal transglutaminase, rather than tissue transglutaminase, is the dominant autoantigen in dermatitis herpetiformis and explain why skin symptoms appear in a proportion of patients having gluten sensitive disease.

Original languageEnglish
Pages (from-to)747-757
Number of pages11
JournalJournal of Experimental Medicine
Volume195
Issue number6
DOIs
Publication statusPublished - Mar 18 2002

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Keywords

  • Celiac disease
  • Gluten sensitive enteropathy
  • IgA
  • Immune complex
  • Skin

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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