An unusual case of Sturge-Weber syndrome in association with phakomatosis pigmentovascularis and Klippel-Trenaunay-Weber syndrome.
An unusual case of Sturge-Weber syndrome in association with phakomatosis pigmentovascularis and Klippel-Trenaunay-Weber syndrome.
复制标题
Sturge-Weber 综合征与色素性血管性色素瘤病和 Klippel-Trenaunay-Weber 综合征相关的罕见病例。
DOI：
10.1097/00006982-200206000-00022
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发表时间：
2002
期刊：
影响因子：
--
通讯作者：
I. Ziraman
中科院分区：
文献类型：
--
作者：
M. S. Sarıcaoğlu;D. Güven;A. Karakurt;A. Sengun;I. Ziraman
斯特奇-韦伯综合征(SWS)的定义是面部葡萄酒色斑、软脑膜血管异常和与青光眼相关的脉络膜血管病变。1系统性红斑狼疮与色素血管沉着症(鲜红斑痣、真皮黑素细胞增多症、雀斑或咖啡斑的相关性)的关联很少见，已有少数病例报道。1-3在SWS中，脉络膜血管瘤经常累及面部鲜红斑痣同侧眼，4双侧脉络膜血管瘤合并双侧鲜红斑痣是一种罕见的情况。最近，报道了一例罕见的双侧弥漫性脉络膜血管瘤合并单侧面部鲜红斑痣的病例。5在此病例中，我们报告一例罕见的系统性红斑狼疮，合并双侧脉络膜血管瘤、太田痣、面部鲜红斑痣及单侧上肢血管瘤合并肥大。
Sturge–Weber syndrome (SWS) is defined by the presence of a facial port-wine stain, leptomeningeal vascular anomalies, and choroidal vascular lesions associated with glaucoma. 1 The association of SWS with phakomatosis pigmentovascularis (the association of nevus flammeus, dermal melanocytosis, and nevus spilus or cafe-au-lait spots) is infrequently seen, and a few cases have been reported. 1–3 In SWS, choroidal hemangiomas occur with frequent involvement of the eye ipsilateral to the facial nevus flammeus, 4 and the presence of bilateral choroidal hemangioma in association with bilateral facial nevus flammeus is a rare condition. Recently, a rare case with a combination of bilateral diffuse choroidal hemangioma and unilateral facial nevus flammeus in SWS has been reported. 5In this case report, we present an unusual case of SWS with bilateral choroidal hemangioma, nevus of Ota, facial nevus flammeus, and unilateral upper limb angioma in association with hypertrophy.