64例天疱疮临床分析

A Clinical Analysis of 64 Cases of Pemphigus

目的探讨天疱疮合理的治疗方案以及与其预后有关的因素。方法对1986～1996年间本院64例天疱疮住院患者的临床资料作回硕性分析。结果 64例患者中62例经皮质类固醇激素治疗后皮损得到控制,其中12例轻型患者平均强的松控制剂量为0.958 mg·kg^(-1)·d^(-1);50例重型患者平均强的松控制剂量为1.145 mg·kg^(-1)·d^(-1)。62例中26例单用激素治疗,36例激素并用免疫抑制刺治疗,两者的平均强的松控制剂量分别为1.072及1.178 mg·kg^(-1)·d^(-1);副作用的发生率分别为15.4%及36.1%。8例死亡患者中6例是寻常型天疱疮,7例病程在3年以内。结论天疱疮治疗首选中、大剂量皮质类固醇激素,激素治疗有效者不必并用免疫抑制剂。寻常型天疱疮预后较差,发病后能活4年以上者预后较好。

Reasonable therapeutic dosage regimen for pemphigus are analyzed to find the factors related to prognosis. Methods A retrospective study is made on 64 cases of pemphigus who were admitted to our hospital between 1986～1996. Results Sixty-two of 64 cases with pemphigus were treated with systemic administration of corticosteroids. The average dosage of prednisone for controlling the diseases were 0.958 mg·kg^(-1)·d^(-1) for mild-type of pemphigus (12 cases) and 1.145 mg·kg^(-1)·d^(-1) for severe-type (50 cases) respectively. Furthermore, 26 of 62 cases were treated with corticosteroids only, and the average dosage of prednisone for controlling the diseases was 1.072 mg·kg^(-1)·d^(-1) while in 36 cases treated with corticosteroids combined with immunosuppressors, the average dosage of prednisone was 1. 178 mg·kg^(-1)·d^(-1). The rate of side-effects and complications were 15.4% in patients with corticosteroids alone and 36.1% in patients with combined therapy. Six of eight patients who died were diagnosed pemphigus vulgaris and seven with a course of disease less than 3 years. Conclusions We recommend a moderate to high dosage of corticosteroids be the first choice to control pemphigus. Those who were effective with steroids are not recommended to use additional immunosuppressors. Patients with pemphigus vulgaris usually have a poor prognosis while those who could survived more than 4 years usually have a better out come.