Ann Dermatol Vol. 23, No. 4, 2011 CASE REPORT Sturge-Weber Syndrome http://dx.doi.org/10.5021/ad.2011.23.4.551 Sturge-Weber Syndrome Ling Chen, M.D., Jinjin Wu, M.D., Ph.D., Minhui Xu, M.D., Ph.D.1, Nian Chen, M.D., Yadong Yang, M.D., Ph.D. Departments of Dermatology and 1Neurosurgery, Daping Hospital, Third Military Medical University, Chongqing, China Sturge-Weber syndrome (SWS) is a neurocutaneous syn- drome, characterized by the association of facial port-wine hemangiomas in the trigeminal nerve distribution area, with vascular malformation(s) of the brain (leptomeningeal angioma) with or without glaucoma. Herein, we reported Sturge-Weber syndrome in a 50-year-old man, who pre- sented port-wine hemangiomas and epilepsy. In this case, the patient’s epilepsy episodes from his first year of life had been ignored and separated from the entity of SWS by his physicians, which led to delayed treatment. This case illustrates the importance of careful examination of patients of any age with hemangiomas in the trigeminal nerve with concomitant episodes of epilepsy. In such cases, there should be yearly neuroimaging screenings to guaranteed early interdisciplinary interventions from the time of definite diagnosis. (Ann Dermatol 23(4) 551∼553, 2011) -Keywords- Epilepsy, Port-wine hemangioma, Sturge-weber syndrome, INTRODUCTION Sturge-Weber syndrome (SWS, also called encephalofacial or encephalotrigeminal angiomatosis) is a neurocutaneous syndrome, characterized by the association of facial port-wine hemangiomas in the trigeminal nerve distri- Received February 22, 2011, Revised April 14, 2011, Accepted for publication May 30, 2011 Corresponding author: Jinjin Wu, M.D., Department of Dermatology, Daping Hospital, Third Military Medical University, Chongqing 400042, China. Tel: 86-23-68757591, Fax: 86-23-68757591, E-mail: wjjjj@163.com This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. bution area, with a vascular malformation of the brain (leptomeningeal angioma) with or without glaucoma1. Although facial hemangiomas in SWS are typical at birth, they are prone to misdiagnosis as haplo-hemangiomas, if other concomitant symptoms are not considered together, which may lead to delayed diagnosis and intervention. CASE REPORT This 50-year-old man was admitted to Daping Hospital for evaluation and aesthetic treatment of left facial port-wine nodules, which presented as port-wine stains at birth. With his increasing age, the stains darkened in color and became raised and thickened. His family complained of his episodes of epilepsy, which started during the first year of his life. He had been taking phenobarbital to control the epilepsy; however, the epilepsy became more intense. On examination, well-demarcated cutaneous port-wine macular stains and vascular nodules were observed on the left side of the face that followed the trigeminal nerve distribution area (Fig. 1A). Hemiatrophy of his right leg and failure of closing together of the right fingers were observed. There was no evidence for either contralateral hemiplegia or intellectual disability. A skin biopsy from one of the angiomatous nodules showed a cavernous hemangioma pattern, and dilated and ectatic thin-walled vessels in the superficial dermis were observed. Immunohistochemistry results (immunoperoxidase method) showed CD34(＋), CD31(＋), and Ki67(−) (Fig. 2). There were no abnormalities related to his right eye, and his left visual field was blocked by the port-wine nodules. Radiologically, homolateral cerebral atrophy and subcor- tical calcifications in the left occipital lobe and leptome- ningeal vascular angiomas located in the parietooccipital area were indicated on computed tomographic (CT) scans, magnetic resonance imaging (MRI), and cerebral CT angiography, respectively (Fig. 1B, C). The diagnosis of Vol. 23, No. 4, 2011 551