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疱疹样皮炎

[Dermatitis herpetiformis].

作者信息

Juratli H A, Görög A, Sárdy M

机构信息

Klinik für Dermatologie und Allergologie, Universitätsklinikum Gießen und Marburg, Standort Marburg, Baldingerstr. 1, 35043, Marburg, Deutschland.

Klinik für Dermatologie, Venerologie und Dermatoonkologie, Fakultät für Medizin, Semmelweis Universität, Budapest, Ungarn.

出版信息

Hautarzt. 2019 Apr;70(4):260-264. doi: 10.1007/s00105-019-4378-8.

Abstract

Dermatitis herpetiformis (DH) is a genetically determined, gluten sensitive autoimmune bullous dermatosis related to celiac disease in which granular, insoluble aggregates in the papillary dermis of epidermal transglutaminase (TG3), immunoglobulin A (IgA), and fibrinogen are present. Detection of the dermal IgA-TG3 immune complex is the gold standard of diagnosis. DH develops in a subpopulation of patients with gluten sensitive enteropathy, characterized by itching, erythematous, excoriated papules showing characteristic distribution over the knees, elbows and buttocks; vesicles are rarely seen. The primary therapy of DH is a strict, lifelong gluten-free diet, and it may be necessary to temporarily give dapsone in case of severe symptoms.

摘要

疱疹样皮炎(DH)是一种由基因决定的、对麸质敏感的自身免疫性大疱性皮肤病,与乳糜泻相关,其表皮转谷氨酰胺酶(TG3)、免疫球蛋白A(IgA)和纤维蛋白原在乳头真皮层中形成颗粒状、不溶性聚集体。检测真皮IgA-TG3免疫复合物是诊断的金标准。DH在患有麸质敏感肠病的患者亚群中发病,其特征为瘙痒、红斑、抓痕丘疹,在膝盖、肘部和臀部有特征性分布;水疱很少见。DH的主要治疗方法是严格的终身无麸质饮食,严重症状时可能需要临时给予氨苯砜。

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