本文报告10例结节性硬化症,男4例,女6例,平均年龄32岁。临床表现以皮脂腺瘤、叶状白斑、鲨鱼皮样斑片、甲下纤维瘤、癫痫与智力低下为主要特征。2例做了CT扫描与手指X线检查,均呈阳性体征。本病为常染色体显性遗传。最后,就本病的病理、临床特征与治疗问题进行文献复习,并加以讨论。 This article reports 10 cases of tuberous sclerosis, 4 males and 6 females, with an average age of 32 years. Clinical manifestations of sebaceous adenoma, leaf leukoplakia, shark plaque, hypothyroidism, epilepsy and mental retardation as the main feature. 2 cases did a CT scan and finger X-ray examination, were positive signs. The disease is autosomal dominant. Finally, the pathological, clinical features and treatment of the disease literature review, and to be discussed.