类天疱疮60例回顾性分析

A retrospective study of sixty hospitalized patients with pemphigoid

目的：探讨类天疱疮的临床分型标准及比较合理的治疗方案，了解类天疱疮与其它疾病之间的关系。方法：回顾性分析了60例类天疱疮住院患者，包括患者发病年龄、病程、住院日、皮损分布、组织病理、直接和间接免疫荧光、常规实验室检查、站疗方案、合并症及并发症等。结果：泛发型类天疱疮和局限型类天疱疮在临床、病理表现、实验室检查及治疗所用皮质类固醇剂量等方面均有显著不同。泛发型组55.6％（25/45）发生粘膜损害，14．29％（5／45）合并恶性肿瘤。治疗本病以系统用皮质类固醇（泼尼松）为主，初始日剂量泛发型组为49．22±13．94mg，局限型组为27．67±16.35mg（P＝0.000001）；激素最大量分别为65．82±36.13mg和34±20.28mg（P=0.001）。使用最大剂量的维持时间两组之间无显著性差异。但是两组所用维持量不同（分别为23．45mg日和13．5mg／日，P＝0．0292）。结论：类天疱疮病情严重程度差异较大，局限型和泛发型类无疱疮的临床和试验室特点以及治疗方案等方面均不相同。

Objectives:To expore the clinical classilieation criteria of pemghgoid, its reasonable therapy regimen as well as relationship between pemphigoid and other diseases. Methods: The study recruited sixty in-patients with pemphigoid of PUMC hospital in the period of 1980~1998. A retrospective research was designed to age at onset of disease, disease duration, number of days for hospitalization,distribution of lesions, lesions, histopathological findings,results of direct and indirect immunofluorencent assays,results of routine laboratory examinations, treatment regimen and complications of the disease. Results:There was significant differences in the aspects of clinical features, histopathological findings, laboratory examination results and the dosage of corticosteroids applied for treatment between dissminat ed and localized two types of pemphigoid. Among 45 cases with disseminated pemphigoid, 25 cases (55.6% ) presented mucosa involvement all 5(14.29%) with malignant tumors. Corticoteriods (prednisone) was the drug of the first choice for the treatment of the disease. For disseminded and localized types, the starting dose (prednisone) was 49.22 ±13.94 mg and 27.67 ±16.35 mg (P = 0.000001) and the maximun dose was 65.82± 36. 13 mg vs 34±20.28 mg (P = 0.001), respectively with a significant difference.The maintenance duration of maximun dose used for two types are similar, there was no significant difference, but the maintenance dase of prednisone used for each type was different (23 .45 mg/d vs 13 .5mg/d, P = 0.0292). Conclusions: Pemphigoid could be classified as two types, disseminated and localized, based on its different clinical and laboratory grounds. The treatment regiment should be selected according to its classification.