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结节性硬化症67例临床分析
引用本文:孙新芬,闫春林,方丽,廖康煌.结节性硬化症67例临床分析[J].临床皮肤科杂志,2005,34(2):78-81.
作者姓名:孙新芬  闫春林  方丽  廖康煌
作者单位:复旦大学华山医院皮肤科,上海,200040
摘    要:目的:了解结节性硬化症(tuberoussclerosis,TS)的特征性皮肤表现及常见的皮肤外病变,使该疾病及内脏病变得到早期诊断、早期发现。方法:回顾性分析67例TS患者的临床、实验室及辅助检查资料。结果:67例TS患者常常受累的器官为皮肤、脑及肾脏,神经系统损害多见,头颅CT检查阳性率高达94.3%。皮肤损害最常见和最早出现的表现是色素减退斑,有81.5%的患者其色素减退斑数目>3个,其次常见的是面部血管纤维瘤和鲨革样皮疹;前额纤维斑块、面部血管纤维瘤、鲨革样皮疹、甲周纤维瘤平均发现年龄分别为2.2、5.7、7.7、14.5岁。结论:临床医师对色素减退斑应提高警惕,尤其是伴有癫的患者,或者在婴儿期其色素减退斑数目>3个者;头颅CT检查对本病的诊断有帮助。

关 键 词:硬化症,结节性  临床分析
文章编号:1000-4963(2005)02-0078-04
修稿时间:2004年5月8日

Clinical analysis of 67 patients with tuberous sclerosis
SUN Xin-fen,YAN Chun-lin,FANG Li,LIAO Kang-huang.Clinical analysis of 67 patients with tuberous sclerosis[J].Journal of Clinical Dermatology,2005,34(2):78-81.
Authors:SUN Xin-fen  YAN Chun-lin  FANG Li  LIAO Kang-huang
Abstract:Objective: To study the clinical features of tuberous sclerosis(TS) and help the clinicians diagnose correctly and detect important visceral involvement early. Methods: The clinical data of 67 patients with TS were analyzed retrospectively, including detailed history, physical and dermatological examination, cranial computerized tomography(CT) scanning and magnetic resonance imaging(MRI), abdominal ultrasonography, chest roentgenogram, hand and foot X-ray and ophthalmologic examination. Results: The results showed that the skin, brain and kidney were involved frequently in TS patients. The hypopigmented macule was usually the most common and earliest cutaneous manifestation, of which the number was more than 3 in 81.5% patients,and the next were facial angiofibromas and shagreen patch. The mean ages of appearance of facial angiofibromas,shagreen patch, forehead plaque and periungual fibromas were 2.2?5.7?7.7?14.5 years old respectively. The involvement of nervous system was very common in TS patients. Cranial CT scanning of TS patients had a high positive rate (94.3%). Conclusions: The cutaneous features are helpful in the early diagnosis of TS. The involvement of visceral organs such as brain and kidney should be examined and followed up in TS patients. The clinicians should notice the hypopigmented macules in the patients especially with epilepsy or if the number is more than 3 during infancy. Cranial CT scanning is of great value in the diagnosis of TS.
Keywords:sclerosis  tuberous  clinical analysis  
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