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家族性良性慢性天疱疮40例临床分析
引用本文:田洪青,颜潇潇,张福仁,周桂芝,卢宪梅,王昌媛.家族性良性慢性天疱疮40例临床分析[J].中国麻风皮肤病杂志,2006,22(4):277-279.
作者姓名:田洪青  颜潇潇  张福仁  周桂芝  卢宪梅  王昌媛
作者单位:山东省皮肤病性病防治研究所,济南,250022
摘    要:目的分析40例山东地区家族性良性慢性天疱疮患者的临床表现、加重因素、病程和治疗等方面特征。方法对17个先证者及其家系患者共40例的临床资料进行了分析。结果(1)发病年龄多在20~40岁,好发于腋窝、腹股沟等皱褶部位;(2)食物、药物、心理等因素可以加重病情;(3)4例患者有乏力的表现,与皮疹严重程度一致;(4)病程超过20年的患者中,多数(10/15)无逐年好转的趋势;(5)对药物治疗的反应个体差异较大,局部结合系统治疗有一定疗效。结论家族性良性慢性天疱疮病程长,有一定加重因素和临床特征,乏力可能是该病的全身症状,阿维A与雷公藤联合应用效果好。

关 键 词:家族性良性慢性天疱疮  临床分析
收稿时间:2005-08-04
修稿时间:2005-11-11

Clinical analysis of forty patients with Hailey- Hailey disease
Tian Hongqing, Yah Xiaoxiao, Zhang Furen,et al..Clinical analysis of forty patients with Hailey- Hailey disease[J].China Journal of Leprosy and Skin Diseases,2006,22(4):277-279.
Authors:Tian Hongqing  Yah Xiaoxiao  Zhang Furen  
Institution:Department of Dermatology, Shandong Provincial Institute of Dermatology and Venerology, Jinan, 250022
Abstract:Objective: To analyze the clinical features, aggregative factors, the course and treatment response in 40 patients with Hailey-Hailey disease. Methods: The clinical data of 40 patients from17 families were analyze. Results: (1) In majority of patients, the onset of the disease started in their 20-40 decade. The skin lesions were mainly located in the groins and axillae. (2) Food, drug and mood might exacerbate the disease. (3) Fatique consistenting with the severity of skin lesions was systemic sign of some patients. (4) Majonty of patients (10/15) with a course of disease longer than 20 years didn't show improvement as ageing. (5) Individuals variably respond to the medicine, and combined treatment with oral medicine and Lei gong Teng was quite effective to some of the patients. Conclusion : Hailey-Hailey disease with clinical feature of skin rush can present with systemic manifestation of fatigue. It may lasts for lifelong time in some patients. Some factors might exacerbate the disease. Combined treatment of acitretin and tripterygium wicfordi was effective to the patients.
Keywords:familial benign chronic pemphigus  clinical analysis  
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