弥漫性躯体性血管角皮瘤  

Fabry disease:a case report

作　　者：辛崇美[1] 徐秀莲[1] 孙建方[1] 

机构地区：[1]中国医学科学院、北京协和医学院皮肤病研究所,江苏南京210042

出　　处：《临床皮肤科杂志》2010年第4期227-228,共2页Journal of Clinical Dermatology

摘　　要：报告1例弥漫性躯体性血管角皮瘤(Fabry病)。患儿男,14岁。全身暗红色丘疹伴皮肤干燥3年。体格检查见躯干、四肢等部位数百枚针尖至米粒大的暗红色斑疹、丘疹,阴囊部位见粟米大小暗红色丘疹,高出皮面,压之不退色,躯干、四肢皮肤干燥。皮损组织病理检查:真皮乳头层见扩张的毛细血管,内皮细胞、周细胞和成纤维细胞的胞质内见脂质空泡。A 14-year-old boy with angiokeratoma is reported. He had dark red papules presented on the trunk, limbs and scrotum accompanied by xeroderma for 3 years. Physical examination showed there were hundreds of clark red papules scattered on the abdomen, buttocks, flanks, penis and scrotum. Histopathology showed the dilated vessels and the accumulated glycolipid deposition in the vascular endothelial, perithelial cells and fihroblasts.

关 键 词：血管角皮瘤 躯体性 弥漫性 

分 类 号：R758.5[医药卫生—皮肤病学与性病学]