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目的分析儿童结节性硬化症皮损与疾病过程的关系,提高早期诊断率。方法回顾性分析25例儿童结节性硬化症的临床资料。结果 25例均有皮肤表现,包括色素脱失斑(24/25)、面部血管纤维瘤(15/25)、牛奶咖啡斑(13/25)、鲨鱼皮斑(9/25)、头部纤维性斑块(10/25)、指(趾)甲纤维瘤(2/25)、口内纤维瘤(2/25)。15例出生时即有皮损,其中色素脱失斑6例、牛奶咖啡斑1例、色素脱失斑和牛奶咖啡斑同时存在4例、色素脱失斑和头部纤维性斑块同时存在2例、指(趾)甲纤维瘤2例。面部血管纤维瘤在1~4岁出现,青春期时增多增大。结论结节性硬化症在婴幼儿期或胎儿期即可发病。大多数皮损有各自的发病年龄和发展规律。色素脱失斑是最早和最常见的皮肤表现,早期发现3处以上时应高度怀疑本病。
Objective To analyze the relationship between childhood tuberous sclerosis and the disease process, and to improve the early diagnosis rate. Methods The clinical data of 25 children with tuberous sclerosis were retrospectively analyzed. Results All the 25 cases had skin manifestations, including pigmented plaque (24/25), facial fibroadenoma (15/25), milk coffee spot (13/25), shark skin spot (9/25) Sexual plaques (10/25), nail fibroids (2/25), intraoral fibroids (2/25). 15 cases had skin lesions at birth, including 6 pigmented lesions, 1 coffee milk stain, 4 pigmented spots and milk coffee spots, 2 pigmented spots and fibrous head plaques.2 Cases, finger (toe) two cases of fibroma. Facial vascular fibroids in 1 to 4 years of age, increased adolescence increased. Conclusion Tuberous sclerosis can develop in infancy or fetus. Most lesions have their own age of onset and development. Pigment depigmentation is the earliest and most common skin manifestations, early detection of more than 3 should be highly suspected of the disease.