Vascular abnormalities in epidermal nevus syndrome.

We report a patient with epidermal nevus syndrome and right hemispheric infarct and review 3 others with neurologic manifestations best explained by ischemia or hemorrhage. Each had a significant vascular abnormality such as occlusion or blood vessel dysplasia. None had hemimegalencephaly. We hypothesize that underlying vascular dysplasia is the cause of the neurologic lesions in these patients.