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Updated: Jan 24, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
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Fibrillar-type dermatitis herpetiformis.

Teruhiko Makino1, Tadamichi Shimizu1

  • 1Department of Dermatology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama.

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|May 21, 2019
PubMed
Summary

Dermatitis herpetiformis (DH) presents differently in Japan, with a "fibrillar-type" showing unique IgA deposition and rare gluten-sensitive enteropathy (GSE). This contrasts with the common "granular-type" DH seen in Caucasians.

Keywords:
dermatitis herpetiformisfibrillar IgA depositionfibrillar-type DHgluten-sensitive enteropathygranular-type DH

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Area of Science:

  • Immunodermatology
  • Gastroenterology
  • Genetics

Background:

  • Dermatitis herpetiformis (DH) is a pruritic disease common in Caucasians, linked to gluten-sensitive enteropathy (GSE) and HLA-DQ2/DQ8.
  • DH is rare in Japan, where unique clinical and immunological features are observed in affected patients.
  • Japanese DH patients often exhibit fibrillar IgA deposition and infrequent GSE, distinct from the typical granular IgA pattern in Caucasians.

Purpose of the Study:

  • To review the clinical, histological, and immunological characteristics of fibrillar-type DH observed in Japanese patients.
  • To differentiate fibrillar-type DH from the granular-type DH prevalent in Caucasian populations.
  • To explore potential differences in pathogenesis between these DH subtypes.

Main Methods:

  • Review of clinical presentations of Japanese DH patients.
  • Histopathological analysis focusing on IgA deposition patterns (granular vs. fibrillar).
  • Immunological investigations including antibody detection (e.g., anti-epidermal transglutaminase) and cytokine profiling (e.g., IL-4, IL-5, IL-13).

Main Results:

  • Japanese DH patients frequently show fibrillar IgA deposition in the papillary dermis, unlike the granular IgA in the dermal-epidermal junction seen in Caucasians.
  • Gluten-sensitive enteropathy (GSE) is rarely associated with fibrillar-type DH.
  • IgA antibodies to epidermal transglutaminase are detected in fibrillar-type DH, and Th2 cytokines may play a role in skin lesion development.

Conclusions:

  • Fibrillar-type DH in Japan represents a distinct entity from the typical granular-type DH found in Caucasians.
  • The pathogenesis of fibrillar-type DH may differ from granular-type DH, potentially involving different triggers and mechanisms.
  • Further research is needed to fully elucidate the distinct pathogenic pathways of these DH subtypes.