Reunala T
Ann Med. 1998 Oct;30(5):416-8. doi: 10.3109/07853899809002482.
Dermatitis herpetiformis (DH) is a lifelong, gluten-sensitive, blistering skin disease with pathognomonic immunoglobulin (Ig)A deposits in the papillary dermis. Less than 10% of patients with DH have gastrointestinal symptoms suggestive of coeliac disease, yet they all have gluten-sensitive enteropathy. The rash, too, responds to gluten withdrawal. Therefore, DH provides a unique example of the frequent existence of gastroenterologically 'silent' but dermatologically active coeliac disease. DH and coeliac disease are strictly associated with class II HLA alleles A10501 and B102 encoding the HLA-DQ2 heterodimer. Coeliac disease segregates in the families of patients with DH, also supporting a shared genetic background. Monozygotic twins, one with DH and the other with coeliac disease, show that environmental, not genetic, factors seem to be responsible for the development of the rash in DH. A clinically silent but immunologically active coeliac disease in the gut could well produce IgA autoantibodies which react also with the connective tissue in the skin. The antigen for deposited IgA and its role, if any, in the blister formation in DH remains, however, to be elucidated.
疱疹样皮炎（DH）是一种终身性、对麸质敏感的水疱性皮肤病，在乳头真皮层有特征性的免疫球蛋白（Ig）A沉积。不到10%的DH患者有提示乳糜泻的胃肠道症状，但他们都有麸质敏感性肠病。皮疹也会因戒食麸质而消退。因此，DH提供了一个独特的例子，说明在胃肠方面无症状但在皮肤方面有症状的乳糜泻经常存在。DH和乳糜泻与编码HLA-DQ2异二聚体的II类HLA等位基因A10501和B102密切相关。乳糜泻在DH患者的家族中呈分离状态，这也支持了共同的遗传背景。同卵双胞胎，一个患有DH，另一个患有乳糜泻，这表明环境因素而非遗传因素似乎是导致DH皮疹发生的原因。肠道中临床上无症状但免疫活跃的乳糜泻很可能产生也与皮肤结缔组织发生反应的IgA自身抗体。然而，沉积IgA的抗原及其在DH水疱形成中的作用（如果有）仍有待阐明。
