大疱性类天疱疮89例临床分析

Clinical Analysis of 89 Cases of Bullous Pemphigoid

目的探讨大疱性类天疱疮患者的临床表现、实验室检查和治疗的特点。方法回顾性分析89例大疱性类天疱疮患者的临床资料。结果89例患者,男女之比1.07∶1,平均发病年龄58岁。皮损除典型的水疱、红斑外,还有多形红斑、疱疹样皮炎样损害。33.7%的患者有口腔粘膜损害,6.7%的患者以口腔水疱、糜烂为首发症状。18%的患者尼氏征阳性。间接免疫荧光法检测阳性率74.4%,直接免疫荧光法检测阳性率94.9%。皮质类固醇以及皮质类固醇联合免疫抑制剂是治疗大疱性类天疱疮的主要手段,除接受冲击治疗的患者外,控制皮损所需的皮质类固醇剂量平均值为65.5mg(相当于泼尼松)。结论组织病理和免疫荧光检测是确诊的主要依据,控制皮损的皮质类固醇最大用量存在个体差异。

Objective To study the clinical features, laboratory findings and therapy of patients with bullous pemphigoid. Methods Clinical data of 89 cases of bullous pemphigoid were retrospectively analyzed. Results The male female ratio was 1.07∶1. The mean age of first onset was 58 years old. Patients presented erythema multiforme and dermatitis herpetiformis like lesions besides typical bullae and erythema. Oral mucosal lesions occurred in 33.7% of patients, and oral bullae and erosions were the inital symptoms in 6.7% of patients. Nikolsky′s sign was present in 18% of patients. Direct and indirect immunoflorescence were positive in 95% and 74.4% of patients, respectively. The patients were mainly treated with corticosteroids or the combination of corticosteroids with immunosuppressive agents. The mean dose of coricosteroid needed to control lesions was 65.5 mg (equivalent of prednisone), except patients treated with pulse therapy of corticosteroids. Conclusions Histopathological and immunofluorescent findings are the most important evidence for the diagnosis of bullous pemphigoid. The maximum doses needed to control lesions vary greatly in different individuals.