【摘要】 目的:探讨各种自身免疫性大疱病的临床特征和合适的治疗方法。方法:对135例自身免疫性大疱病进行回顾性分析。结果:天疱疮、类天疱疮和线状IgA大疱性皮病是自身免疫性大疱病中常见的3种疾病,分别易累及中年人、老年人和儿童。寻常型天疱疮伴发口腔黏膜受累多见(73.1%)。在病情得到控制的患者中,重症患者糖皮质激素用量泼尼松(78.7±23.9)mg/d显著高于轻、中症患者泼尼松(45.8±19.8)mg/d和(59.4±20.8)mg/d,P<0.001;寻常型、落叶型天疱疮糖皮质激素用量(66.2±24.3)mg/d、(73.0±14.9)mg/d显著高于红斑型(49.1±21.8)mg/d,P<0.05;寻常型天疱疮痊愈率(24.2%)显著低于红斑型(63.6%),P<0.05。接受相近剂量的糖皮质激素治疗,类天疱疮痊愈率(56.1%)明显高于天疱疮(35.5%),P<0.05。结论:应根据不同情况确定糖皮质激素合适的初始剂量。对于线状IgA大疱性皮病和IgA型大疱性系统性红斑狼疮,氨苯砜是首选治疗药物。更多还原

【Abstract】 Objective: To study clinical features and an appropriate treatment of several kinds of autoimmune bullous diseases. Methods: The clinical data of 135 patients with autoimmune bullous diseases from 1994 to 2003 from 1994 to 2003 were reviewed. Results: The results showed that pemphigus, bullous pemphigoid, and linear IgA bullous dermatoses were common in this group of patients, mainly involving patients with middle-aged and old age, and children respectively. Oral involvement was very common in pemphigus vulgaris (73.1%). The dose of corticosteroids in severe cases predisone (78.7±23.9) mg/d was much higher than that in mild or moderate cases (45.8±19.8) mg/d and (59.4±20.8) mg/d. The dose in pemphigus vulgaris or foliaceus was much higher than that in pemphigus erythematosus. However, the cure rate of the former was much lower than that of the latter. The cure rate of pemphigoid was obviously higher than that of pemphigus after these two diseases were treated with approximate dose of corticosteroids. Conclusion: The suitable initial dose of corticosteroids should be considered according to different conditions. Dapsone should be the first choice in linear IgA bullous dermatoses and IgA mediated systemic lupus erythematosus.更多还原