良性多发对称性脂肪增多症2例报告并文献复习  

Benign Symmetrical Lipomatosis:Two Cases Report and Review of Literatures

作　　者：常子强[1] 李淑娟[1] 

机构地区：[1]中国医科大学北京顺义医院急诊科,101300

出　　处：《中国误诊学杂志》2009年第35期8575-8577,共3页Chinese Journal of Misdiagnostics

摘　　要：目的:提高对良性多发对称性脂肪增多症(Benign symmetrical lipomatosis,BSL,又称Madelung病)的认识。方法:报告2例BSL并复习相关文献。结果:BSL主要表现为面、颈、枕部、上背部、腋下、腹股沟及其他部位多发对称的无包膜的弥漫性脂肪堆积,可导致头颈部畸形及压迫症状。BSL90%以上病例与酗酒有关,无明显的遗传倾向,但确切病因仍不清楚;也有研究表明异常增生的脂肪是由儿茶酚胺引起的。BSL恶变很少见。BSL的诊断主要依靠临床表现和影像学检查。结论:BSL是一种罕见疾病,BSL的治疗主要采用外科手术,其它的治疗如β2受体激动剂病灶内注射也有一定的疗效。Objective：To improve the knowledge of benign symmetrical lipomatosis（BSL）.Methods：Two cases of BSL were reported and the relative literatrues were reviewed.Results：The main clinical manifestation of BSL was characterized by the presence of multiple,symmetric,nonencapsulated fat masses in the face,neck,upper back,hips,deltoid region,and other areas.Most cases had no hereditary pattern.More than 90% of the patients had associated alcoholism.The ethiology of BSL remains unknown,but an abnormal lipogenesis induced by catecholamines had been observed.The transformation of BSL to a malignant tumor was extremely rare.The diagnosis of BSL was established with the clinical dates and image tests.Conclusions：BSL is a rare disorder.The best management of BSL is surgical approach.Other treatments such as β2 agonists injected into focus could have efficacies.

关 键 词：脂肪瘤样病 多发性对称性/诊断/治疗 

分 类 号：R589.2[医药卫生—内分泌]