Dermatitis Herpetiformis (DH) is a rare, blistering skin disease described by Duhring in 1884.[1] DH is strongly associated with the HLA-DQ2 phenotype, a gateway in which dietary gluten reaches inflammatory cells and stimulates an autoimmune process. The etiopathogenesis involves IgA anti-endomysial antibodies directed against tissue transglutaminase (TG); the presumed skin autoantigen is epidermal TG. IgA/TG immune complexes form locally within the papillary dermis leading to neutrophil chemotaxis and degranulation (which forms neutrophilic abscesses), proteolytic cleavage disrupting the lamina lucida, and blister subepidermal blister formation.[2] Current standard-of-care for DH is oral dapsone and a gluten-free diet. We describe a teenage patient in whom resolution of lesions was achieved with adjuvant use of topical dapsone 5% gel (aczone), the first case in the literature.
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机译：皮肤性疱疹样皮肤病（DH）是Duhring在1884年描述的一种罕见的水疱性皮肤病。[1] DH与HLA-DQ2表型密切相关，HLA-DQ2表型是饮食面筋到达炎症细胞并刺激自身免疫过程的途径。致病机理涉及针对组织转谷氨酰胺酶（TG）的IgA抗内膜抗体。假定的皮肤自身抗原是表皮TG。 IgA / TG免疫复合物在乳头状真皮内局部形成，导致嗜中性粒细胞趋化性和脱粒（形成嗜中性脓肿），蛋白水解裂解破坏了透明质层和水疱的表皮下水疱形成。[2] DH的当前护理标准是口服氨苯砜和无麸质饮食。我们描述了一名青少年患者，通过使用局部5％氨苯砜凝胶（aczone）辅助治疗，可解决病变，这是文献中的第一例。
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