六例结节性类天疱疮临床和实验室检查特点

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。

Clinical and Laboratory Features of 6 Cases of Pemphigoid Nodularis

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.