黏膜性类天疱疮68例临床回顾分析

Mucous membrane pemphigoid： a retrospective study of 68 cases

目的总结1987年8月至2012年10月我科诊治的68例黏膜性类天疱疮的临床特点和治疗经验。方法总结黏膜性类天疱疮患者黏膜皮肤损害、组织病理改变、免疫学等方面的特点，重点分析治疗方案。结果黏膜性类天疱疮68例，累及口腔黏膜67例占98．5％，眼部黏膜23例占33．8％。免疫学检查：16例行直接免疫荧光检查（DIF），8例阳性；39例行间接免疫荧光检查（IIF），8例阳性，阳性率20．5％；41例采用ELISA法检测血清中抗BP180抗体，22例阳性，阳性率53．7％。依据皮损分布确定病情严重程度后，予以局部治疗5例；中低剂量糖皮质激素系统治疗（0．4—0．5mg·kg^-1·d^-1，或30mg／d）55例，其中3例同时累及皮肤、口腔黏膜、眼结膜及其他黏膜的患者剂量增加至50mg／d左右才能控制病情。控制剂量起效时间（11．80±5．88）d，控制剂量服药时间0．23～12（3．06±2．84）个月。15例患者基本治愈，其中4例完全治愈。基本治愈患者糖皮质激素剂量减至原剂量50％所需时间为（13．29±5．76）个月，达到基本治愈所需时间为（17．33±7．71）个月。病情控制6个月后，剂量平均减至控制剂量的76．5％，12个月后剂量平均减至控制剂量的58．1％。3例患者在治疗过程中出现口腔念珠菌感染。结论黏膜性类天疱疮的诊断目前主要依据典型的临床和病理表现，尚缺乏灵敏度高的免疫学检查手段。中低剂量糖皮质激素系统治疗，配合局部治疗，常可达满意疗效。

Objective To analyze the clinical features and treatment of 68 cases of mucous membrane pemphigoid （MMP）. Methods Clinical data were collected from 68 patients with MMP at the Department of Dermatology, Peking University First Hospital, between August 1987 and October 2012. Skin manifestations, histopathological and immunological findings were studied, with an emphasis on treatment regimens. Results The two most frequently involved sites were oral （67/68, 98.5%） and conjunctival （23/68, 33.8%） mucosa in patients with MMP. Immunological examinations included direct immunofluorescence test, indirect immunofluorescence test and enzyme-linked immunosorbent assay, with a positivity rate of 50% （8/16）, 20.5% （8/39） and 53.7% （22/41） respectively. According to lesion distribution and disease severity, patients were given local therapy （n = 5） or low to moderate dose of glucocorticoids （n = 55, 0.4 - 0.5 mg/kg or 30 mg per day）. The condition was controlled until the dose of glucocorticoids reached 50 mg/d in three patients with cutaneous, oral, ocular, and other mucosal involvement. The time to onset of action of glucocorticoids at the controlling dose was （ 11.80 ± 5.88） days, and the duration of administration of glucoeorticoids at this dose varied from 0.23 to 12 months （average, 3.06 ± 2.84 months）. Fifteen patients were almost cured, and four patients completely cured. Of the 15 patients almost cured, the time required for a 50% reduction in the dose of glueocortieoids was （13.29 ± 5.76） months,and that required for the control of MMP was （17.33 ± 7.71 ） months. The dose of glucocortieoids was decreased to 76.5% of the controling dose at 6 months after the control of MMP, and 58.1% of that at 12 months. Oral candidiasis occurred in three patients during the treatment. Conclusions The diagnosis of MMP is mainly based on typical clinical and histopathological findings, and current immunological examinations are insufficiently sensitive. Usually, low to moderate dose of systemic corticosteroids combined with topical therapy can lead to satisfactory treatment outcomes.