胸骨畸形/血管发育不良综合征伴线状色素减退  

Sternal malformation/vascular dysplasia syndrome with linear hypopigmentation

作　　者：Mazereeuw-Hautier J. Syed S. Harper J.I. 刘艳 

机构地区：[1]Department of Dermatology, Great Ormond Street Hospital, London WC1N 3JH, United Kingdom

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2006年第9期41-42,共2页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：We report a 7-year-old boy who presented with a facial haemangioma, a circumscribed depression over the sternum, coarctation of the aorta, ventricular septal defect and dysplastic cerebral arteries responsible for an episode of acute infarct. This combination of clinical features has been described as the sternal malformation/vascular dysplasia syndrome or PHACES syndrome. At the age of 5 years, lines of hypopigmentation were noted on the right arm, the right hand and the back, along the lines of, with no history of any preceding inflammatory changes, and have persisted unchanged. These pigmentary changes have not previously been reported in association with this syndrome.We report a 7-year-old boy who presented with a facial haemangioma, a circumscribed depression over the sternum, coarctation of the aorta, ventricular septal defect and dysplastic cerebral arteries responsible for an episode of acute infarct. This combination of clinical features has been described as the stemal malformation/vascular dysplasia syndrome or PHACES syndrome. At the age of 5 years, lines of hypopigmentation were noted on the right arm, the right hand and the back, along the lines of, with no history of any preceding inflammatory changes, persisted unchanged. These pigmentary changes previously been reported in association with this s and have have not yndrome.

关 键 词：发育不良综合征 胸骨畸形 色素减退 血管瘤 线状 BLASCHKO线 主动脉缩窄 室间隔缺损 

分 类 号：R682.1[医药卫生—骨科学]