六例结节性类天疱疮临床和实验室检查特点  

Clinical and Laboratory Features of 6 Cases of Pemphigoid Nodularis

作　　者：盖晓磊 李思哲 左亚刚 杜伟 何春霞 GE Xiaolei;LI Sizhe;ZUO Yagang;DU Wei;HE Chunxia(Department of Dermatology,Translational Medicine Center,National Clinical Research Center for Cutaneous and Immunologic Diseases,PUMC Hospital,CAMS and PUMC,Beijing 100730,China;Graduate School of Hebei North University,Zhangjiakou,Hebei 075000,China)

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院皮肤科,协和医学转化中心,国家皮肤与免疫疾病临床医学研究中心,北京100730 [2]河北北方学院研究生院,河北张家口075000

出　　处：《中国医学科学院学报》2020年第2期197-201,共5页Acta Academiae Medicinae Sinicae

基　　金：国家自然科学基金(81972944);北京市自然科学基金(7192166)。

摘　　要：目的探讨6例结节性类天疱疮临床表现和实验室检查特征。方法回顾性分析2016年1月至2019年8月北京协和医院皮肤科住院部6例结节性类天疱疮患者临床资料。结果结节性类天疱疮主要发生于中老年人,平均发病年龄(58±16)岁。4例嗜酸性粒细胞升高,5例进行了免疫球蛋白E(IgE)检测,均升高,平均(530±672)KU/L。直接免疫荧光均提示基底膜带IgG和/或C3沉积,间接免疫荧光示抗基底膜带IgG抗体阳性,滴度1∶40~1∶320。5例患者抗BP180阳性[(24~85)U/ml,平均(43±26)U/ml],未合并中枢神经系统疾病。6例患者中1例失访,5例可随访的患者中3例出现复发,2例仍口服激素维持治疗。结论结节性类天疱疮发病年龄相对较轻,嗜酸性粒细胞和总IgE升高,抗BP180抗体滴度低,容易复发但合并神经系统疾病较少。Objective To investigate the clinical manifestations and laboratory characteristics of 6 cases of pemphigoid nodularis(PN).Method The clinical and laboratory data of 6 patients with PN admitted to the Department of Dermatology,Peking Union Medical College Hospital from January 2016 to August 2019 were retrospectively analyzed.Results PN mainly occurred in middle-aged and elderly people,with an average age of(58±16)years.Eosinophils were elevated in 4 patients.Immunoglobulin E(IgE)level was(530±672)kU/L in five patients.Direct immunofluorescence showed IgG and/or C3 deposition on basal membrane zone.Indirect immunofluorescence showed positive IgG anti-basement membrane zone,with a titer of 1∶40-1∶320.Enzyme-linked immunosorbent assay showed the anti-BP180 antibodies were positive[24-85 U/ml,average(43±26)U/ml]in 5 patients.None of the patients had neurological disorders.One patient was lost to follow-up.The disease recurred in 3 of 5 patients during the follow-up,and two patients still received maintenance corticosteroids.Conclusions PN mainly occurs in middle-aged and elderly individuals.It is featured by elevated eosinophils and total IgE and relatively low anti-BP180 antibody titers.Recurrence is common but PN is less likely to be associated with neurological diseases.

关 键 词：结节性类天疱疮 嗜酸性粒细胞 免疫球蛋白E 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]