疱疹样天疱疮  

Herpetiform Pemphigus

作　　者：吴冠儒 周生儒 赵肖庆 潘萌 WU Guanru;ZHOU Shengru;ZHAO Xiaoqing;PAN Meng(Department of Dermatology,Shanghai Ruijin Hospital,Shanghai 200025,China)

机构地区：[1]Department of Dermatology,Shanghai Ruijin Hospital,Shanghai 200025,China

出　　处：《中国皮肤性病学杂志》2020年第7期838-841,共4页The Chinese Journal of Dermatovenereology

基　　金：国家自然科学基金重点项目(8173000414);国家自然科学基金面上项目(81673064);上海市教育委员会高峰高原学科建设计划(20152203);上海市优秀学科带头人计划(17XD1402700);上海市青年科技英才扬帆计划(19YF1430200)。

摘　　要：疱疹样天疱疮(HP)是天疱疮的罕见类型,常被认为是落叶型天疱疮(PF)的亚型。其临床特点为躯干及四肢环形红斑基础上的紧张性小水疱,尼氏征阴性,黏膜少有累及,常需与疱疹样皮炎等其他大疱性皮肤病相鉴别。病理表现多变,多见表皮内水疱,嗜酸性粒细胞海绵水肿,炎性细胞浸润,而棘层松解则不多见。直接免疫荧光见IgG和/或C3棘细胞间荧光沉积。循环抗体常认为主要是抗桥粒芯糖蛋白(Dsg)1抗体,少数患者为Dsg3抗体,近年来新的循环抗体如抗桥粒芯胶黏蛋白(Dsc)1及Dsc3等抗体亦被发现。患者通常对砜类药物及激素反应良好,相对其他类型的天疱疮而言对治疗较敏感。Herpetiform pemphigus(HP)is a rare kind of pemphigus,which is generally considered as a subtype of pemphigus foliaceus(PF).It is characterized by clinical feature of annular erythema on the trunk and limbs,accompanied by tensive vesicles.Nikolsky's sign is usually negative.Lesions rarely involve mucosa,and differential diagnosis with dermatitis herpetiformis is always required.Histopathological manifestations are variable,including epidermal blisters,eosinophilic spongiosis and inflammatory cell infiltration.Acantholysis is usually considered to be rare.Direct immunofluorescence shows the intercellular fluorescence deposition of IgG and/or C3.Circulating antibodies are usually anti-desmoglein(Dsg)1 antibodies,while a few patients have anti-dsg3 antibodies.Recent years,new circulating antibodies such as anti-desmocollin(Dsc)1 and Dsc3 have been discovered.Patients usually respond well to sulfones and corticosteroids and are more easily treated than other types of pemphigus patients.

关 键 词：疱疹样天疱疮 临床表现 组织病理 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]