重度大疱性类天疱疮患者64例临床分析

Clinical Analysis of 64 Patients with Severe Bullous Pemphigoid

目的总结重度大疱性类天疱疮(bullous pemphigoid,BP)患者的临床特点及治疗经验。方法回顾性分析64例BP患者的临床表现、实验室检查、治疗与转归、不良反应及并发症。结果78.12%的患者年龄≥60岁,所有患者均表现为红斑、紧张性水疱,并伴有不同程度的瘙痒。组织病理均表现为表皮下水疱。直接免疫荧光检测阳性率100%,其中98.44%的患者C3阳性,57.81%的患者IgG阳性。25.00%的患者出现不良反应,激素冲击组比激素组更容易出现。联合组住院日为(16.50±5.58)d,与激素组(13.07±5.27)d比较差异有统计学意义。激素冲击组皮损控制时间为(10.82±4.38)d,住院日为(21.09±5.65)d,与激素组(3.89±1.89)d和(13.07±5.27)d比较差异有统计学意义。Logistic回归分析显示激素用量、合并感染与住院日呈正相关。结论基底膜带C3线状沉积对大疱性类天疱疮的诊断敏感性高,可作为疑诊BP的首选检查;激素冲击比常规激素治疗更易出现不良反应;联合治疗、激素用量大、合并感染会延长住院日。

Objective To summarize the clinical features and effective treatment of patients with severe bullous pemphigoid.Methods To retrospectively analyze the clinical features,laboratory findings,therapy and prognosis,adverse reactions and complications of 64 cases of severe bullous pemphigoid.Results 78.12%of the patients were≥60 years old.All patients presented with erythema and tension blisters accompanied by varying degrees of pruritus.Histopathological examination revealed subepidermal blisters.Direct immunofluorescence was positive in 100%of patients,C3 was present in 98.44%of patients,IgG was present in 57.81%of patients.Adverse reactions occurred in 25.00%of patients.The incidence of adverse reactions in the glucocorticoid pulse therapy group was significantly higher than that in the glucocorticoid therapy group.The hospital stay in the combined therapy group[(16.50±5.58)days]were significantly longer than that in glucocorticoid group[(13.07±5.27)days].The duration of lesions control[(10.82±4.38)days]and the hospital stay[(21.09±5.65)days]in the glucocorticoid pulse group were significantly longer than that in glucocorticoid group[(3.89±1.89)days and(13.07±5.27)days].Logistic regression analysis showed that there was a positive correlation between glucocorticoid dose,co-infection and hospital stay.Conclusion The linear deposition of C3 in the basement membrane is highly sensitive to the diagnosis of BP,and can be used as the first choice for suspected BP patients.Glucocorticoid pulse therapy is more likely to cause adverse reactions than routine dose of glucocorticoid therapy.Combination therapy,high dose of glucocorticoid,and co-infection may prolong hospital stay.