Dermatitis herpetiformis
Dermatitis herpetiformis
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DOI：
10.1111/j.1365-2133.1990.tb01862.x
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发表时间：
1990-08
影响因子：
10.3
通讯作者：
中科院分区：
文献类型：
--
作者：
疱疹样皮炎（DH）是一种自身免疫性大疱性疾病，它代表了麸质敏感性的皮肤表现，在乳糜泻的背景下。虽然经典型DH的临床特征是在肿胀的基底上出现成组的囊泡，但由于强烈的相关瘙痒，原发性病变通常不存在。相反，许多病例仅表现为糜烂性糜烂，并伴有大量表皮脱落。在乳糜泻中，DH的核心致病机制可能是由针对几种转氨酶之一的免疫球蛋白A类自身抗体介导的。由于这些自身抗体的产生与胃肠道暴露于麦醇溶蛋白（麦醇溶蛋白是谷蛋白的醇溶性部分）直接相关，因此无谷蛋白饮食代表了DH管理方案的基石。在单纯饮食管理难治的病例中，氨苯砜是治疗DH的一线药物，尽管许多其他药物也被报道有效。
Dermatitis herpetiformis (DH) is an autoimmune bullous disease, which represents the cutaneous manifestation of gluten sensitivity, in the setting of celiac disease. Although classical DH is characterized clinically by grouped, vesicles on an erythematous base, primary lesions often are absent owing to the intense, associated pruritus. Instead, many cases present only with erythematous erosions with numerous overlying excoriations. As in celiac disease, the core pathogenic mechanisms of DH are likely mediated by immunoglobulin A class autoantibodies against one of several transglutaminase enzymes. As the production of these autoantibodies is directly correlated with gastrointestinal exposure to gliadin, which is an alcohol-soluble fraction of gluten, a gluten-free diet represents the cornerstone of a DH management regimen. In cases refractory to dietary management alone, dapsone is the first-line agent for the treatment of DH, although many other agents have been anecdotally reported as effective.