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。
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 froml7 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 - Halley 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.
出处
《中国麻风皮肤病杂志》
2006年第4期277-279,共3页
China Journal of Leprosy and Skin Diseases
基金
山东省科技攻关项目(编号:031050116)
关键词
家族性良性慢性天疱疮
临床分析
familial benign chronic pemphigus
clinical analysis
