摘要目的:总结我国疱疹样皮炎患者的疾病特征。方法:检索中国知网、万方和PubMed数据库中自建库至2018年12月公开发表的中国人疱疹样皮炎相关文献，根据纳入及排除标准对临床表现、普通病理、免疫病理特点进行筛选，分析符合诊断标准患者的疾病特征。结果:纳入55例疱疹样皮炎患者，发病年龄（44.9 ± 18.5）岁，男女比例约2.5∶1，平均诊断延迟4.1年。皮损主要表现为红斑基础上的紧张性水疱，分布在臀区、肘部、背部、膝部等部位。39例描述组织病理表现，37例可见表皮下疱，17例存在中性粒细胞浸润。33例描述皮损周围组织直接免疫荧光表现，31例可见IgA颗粒状沉积，23例沉积部位为真皮乳头。39例描述了治疗情况，其中25例使用氨苯砜或氨苯砜联合无谷胶饮食治疗，治疗效果明显，皮损多在1个月内消退。结论:中国疱疹样皮炎主要表现为红斑基础上的紧张性水疱，直接免疫荧光主要表现为IgA颗粒状沉积，病因可能具有遗传学特点，氨苯砜治疗效果良好。

abstractsObjective: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 from establishment of these databases to December 2020. 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.