疱疹样天疱疮

Pemphigus Herpetiformis

疱疹样天疱疮(Pemphigus herpetiformis,PH)是天疱疮的一种亚型,具有和疱疹样皮炎相类似的临床表现,组织病理呈多形性。免疫学方面,PH患者的血清学中可以检测到抗桥粒芯糖蛋白(desmoglein Dsg)和/或抗桥粒芯胶蛋白(desmocollin Dsc)抗体。在临床工作中,PH的诊断较为困难,常被误诊为疱疹样皮炎,IgA型天疱疮或者类天疱疮。在本文中,我们对PH的临床表现、病理表现、免疫学检查结果、诊断与鉴别诊断、治疗及预后等方面进行了综述。

Pemphigus herpetiformis(PH)is considered as a subtype of pemphigus with similar clinical manifestations to dermatitis herpetiformis and pleomorphic histopathological features.The autoantibodies responsible for PH were thought to be against desmoglein(DSG),however in recent years it is found that patients with PH also have autoantibodies against desmocollin(DSG).In clinical work,the diagnosis of PH is difficult,which was often misdiagnosed as dermatitis herpetiformis,IgA pemphigus or pemphigoid.In this short review,we briefly summarized I he clinical manifestations,histologic findings,immunologic findings,differential diagnosis,treatment and prognosis of PH.