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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 understanding of benign symmetrical lipomatosis (BSL). Methods: Two cases of BSL were reported and relevant literature was reviewed. Results: BSL mainly showed plentiful non-enveloped diffuse fat accumulation on the face, neck, occiput, upper back, armpits, groin and other parts, which could lead to head and neck deformity and compression symptoms. More than 90% of cases of BSL are associated with alcoholism, with no obvious genetic predisposition, but the exact etiology remains unclear. Other studies have shown that dyslipidemia is caused by catecholamines. BSL malignant changes are rare. The diagnosis of BSL depends mainly on clinical manifestations and imaging studies. Conclusions: BSL is a rare disease. The treatment of BSL mainly uses surgery. Other treatments such as intracerebroventricular injection of β2 receptor agonist also have certain curative effect.