Abstract

We reviewed all 91 Japanese dermatitis herpetiformis (DH) patients reported over the last 35 years. The male-to-female ratio was 2 : 1. The mean age at onset was 43.8, and 13 years earlier for female patients. More than half of these Japanese DH patients showed granular IgA deposition in the papillary dermis, and another one-third showed fibrillar IgA deposition. The male patients with granular IgA deposition were 10 years older than those with fibrillar deposition. Whereas patients with granular IgA deposition showed typical distribution of the skin lesions, the predilection sites of DH tended to be spared in patients with fibrillar IgA deposition. Only 3 patients had definite gluten-sensitive enteropathy. There was a statistical difference in the frequency of human leukocyte antigen (HLA)-DR9 between the granular group and controls among Japanese. No patients had HLA-DQ2 or -DQ8, which is frequently found in Caucasian DH patients. The absence of HLA-DQ2/DQ8, the inability to identify celiac disease in most cases, the predominance of fibrillar IgA, and the unusual distribution of clinical lesions in Japanese patients suggest that Japanese DH may be a subset of DH patients and have a pathogenesis which is different from that currently proposed in Caucasian DH patients.

Highlights

  • Dermatitis herpetiformis (DH) is a rare, intensely pruritic, chronic and recurrent papulovesicular disease, in which the lesions usually develop symmetrically on the extensor surfaces

  • The absence of human leukocyte antigen (HLA)-DQ2/DQ8, the inability to identify celiac disease in most cases, the predominance of fibrillar IgA, and the unusual distribution of clinical lesions in Japanese patients suggest that Japanese dermatitis herpetiformis (DH) may be a subset of DH patients and have a pathogenesis which is different from that currently proposed in Caucasian DH patients

  • The absence of HLA-DQ2/DQ8, the inability to identify celiac disease (CD) in most cases, the predominance of fibrillar IgA, and the unusual distribution of clinical lesions in Japanese patients suggest that Japanese DH may be a subset of DH patients and have a pathogenesis which is different from that currently proposed in Caucasian DH patients

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Summary

Introduction

Dermatitis herpetiformis (DH) is a rare, intensely pruritic, chronic and recurrent papulovesicular disease, in which the lesions usually develop symmetrically on the extensor surfaces. This disease can be clearly distinguished from other subepidermal blistering diseases by histopathological and immunological criteria. Biopsy of an early lesion shows collections of neutrophils at the papillary tips, and direct immunofluorescence (DIF) reveals nonlinear (mostly granular, or fibrillar) IgA deposition in the papillary dermis. The previous review of Japanese DH cases revealed differences from Caucasian DH, such as a high frequency of fibrillar IgA deposition in the papillary dermis, a rarity of GSE, and the absence of HLAB8/DR3/DQ2 haplotype [59]

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