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May 1981

The Sturge-Weber SyndromeA Patient With Unusual Features

Arch Dermatol. 1981;117(5):305-306. doi:10.1001/archderm.1981.01650050061023

The Sturge-Weber syndrome is an acquired disorder in which vascular malformations are found in the skin, meninges, and eyes.1 Patients usually have a unilateral nevus flammeus, ipsilateral meningeal angiomatosis, cerebral calcification, and epilepsy. Less frequently, mental retardation, hemiplegia, and congenital glaucoma are found.1-3 A patient who has this syndrome, but with some unusual features, is described.

Report of a Case  The patient, a 53-year-old man, was born with extensive cutaneous vascular nevi. Initially, the nevi were macular, but, with time, cavernous changes developed in some areas. The left eye was involved and was blind from birth. Focal epilepsy commenced in early childhood but was controlled with anticonvulsants. Atrophy and weakness of the right side of the body became apparent as the patient grew older. Despite these handicaps, he was able to lead a fairly normal life. He did not have any children. There was no family history of