TY - JOUR A2 - Caproni, Marzia AU - Ohata, Chika AU - Ishii, Norito AU - Hamada, Takahiro AU - Shimomura, Yutaka AU - Niizeki, Hironori AU - Dainichi, Teruki AU - Furumura, Minao AU - Tsuruta, Daisuke AU - Hashimoto, Takashi PY - 2012 DA - 2012/06/12 TI - Distinct Characteristics in Japanese Dermatitis Herpetiformis: A Review of All 91 Japanese Patients over the Last 35 Years SP - 562168 VL - 2012 AB - 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. SN - 2314-8861 UR - https://doi.org/10.1155/2012/562168 DO - 10.1155/2012/562168 JF - Clinical and Developmental Immunology PB - Hindawi Publishing Corporation KW - ER -