关键词: 疱疹样皮炎, 水疱, 免疫组织化学, 免疫球蛋白A, HLA抗原, 氨苯砜

Abstract: 【Abstract】 Objective To summarize characteristics of dermatitis herpetiformis（DH）patients in China. Methods Published literature on DH in Chinese patients was searched in China National Knowledge Infrastructure database, Wanfang data and Pubmed database. Patients were screened according to the inclusion and exclusion criteria, and characteristics of patients meeting the diagnostic criteria were analyzed. Results A total of 55 patients with DH were involved in this study. Among these patients, the average age of onset was 44.9 ± 18.5 years, the ratio of males to females was about 2.5∶1, and the average period of delayed diagnosis was 4.1 years. Skin lesions mainly manifested as tense blisters on an erythematous base, and were distributed on the buttocks, elbows, back, knees, etc. Histopathological examination of 39 cases showed subepidermal blisters in 37, and neutrophil infiltration in 17. Direct immunofluorescence study of perilesional skin tissues of 33 cases showed granular IgA deposition in 31, and IgA deposits limited to the dermal papilla in 23. Among 39 cases with available data on treatment, 25 were treated with dapsone alone or in combination with a gluten-free diet, most of whom achieved marked therapeutic outcomes with subsidence of skin lesions within 1 month. Conclusions DH in China mainly clinically manifests as tense blisters on an erythematous base, and is mainly characterized by granular IgA deposition under a direct immunofluorescence microscope. Genetic factors are involved in its etiology, and dapsone is effective for its treatment.

Key words: Dermatitis herpetiformis, Blister, Immunohistochemistry, Immunoglobulin A, HLA antigens, Dapsone