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大疱性类天疱疮:疾病进程。对25例患者的研究。

Bullous pemphigoid: course of the disease. A study of 25 patients.

作者信息

Carro-Jiménez Eric J, Sánchez Jorge L

机构信息

Department of Dermatology, University of Puerto Rico, School of Medicine, PO Box 365067, San Juan, Puerto Rico 00936-5067.

出版信息

Bol Asoc Med P R. 2003 Jul-Aug;95(4):27-8, 33-5.

Abstract

Different studies on the course of bullous pemphigoid have suggested that there may be significant variations in the survival of these patients based on their ethnicity or region of origin. Because of the lack of studies on patients with bullous pemphigoid in the Caribbean, specifically in Puerto Rico, a retrospective analysis about the outcome of patients with this disease in the region was performed. Twenty-five (25) patients were included in this study. The patients were diagnosed as bullous pemphigoid by clinical, histopathologic and immunopathologic assessment. Medical records were reviewed and those patients that were alive were contacted and examined on a follow up visit. Nine were men and sixteen were women; the age at the time of the diagnosis ranged from 54 to 90 year-old (mean 72); 13 were alive after at least 5 years of follow-up and 12 had died. The age at the moment of death ranged from 67 to 95 year-old (mean 83). Of those patients living, 85% were in remission, while 15% had active disease. In general, patients had a disease of mild severity, but, still, 68% of them received systemic corticosteroid therapy. Five of patients had to be admitted due to disease; two of them died while at the hospital. This data suggests that bullous pemphigoid in Puerto Ricans is a disease with a relatively benign course, but which can be fatal in the elderly, especially, if it requires admission to the hospital and the patient receives high doses of systemic corticosteroids.

摘要

关于大疱性类天疱疮病程的不同研究表明,这些患者的生存率可能因其种族或原籍地区而存在显著差异。由于加勒比地区,特别是波多黎各缺乏对大疱性类天疱疮患者的研究,因此对该地区这种疾病患者的预后进行了回顾性分析。本研究纳入了25名患者。通过临床、组织病理学和免疫病理学评估将这些患者诊断为大疱性类天疱疮。查阅了病历,并对那些存活的患者进行了随访联系和检查。9名男性,16名女性;诊断时的年龄范围为54至90岁(平均72岁);至少随访5年后,13人存活,12人死亡。死亡时的年龄范围为67至95岁(平均83岁)。在那些存活的患者中,85%处于缓解期,而15%患有活动性疾病。总体而言,患者病情严重程度较轻,但仍有68%的患者接受了全身糖皮质激素治疗。5名患者因病情需要住院;其中2人在医院死亡。这些数据表明,波多黎各人的大疱性类天疱疮是一种病程相对良性的疾病,但在老年人中可能是致命的,特别是如果需要住院且患者接受高剂量全身糖皮质激素治疗的情况下。

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