Leptomeningeal angiomatosis accompanied by hair follicle nevus.

A 5-day-old male infant with leptomeningeal angiomatosis accompanied by hair follicle nevus and congenital alopecia is reported. Admitted for frequent left hemiconvulsions, he had three small papular lesions around his right eye and ipsilateral alopecia from the frontal to parietal areas. Histopathological examination of the papular lesions revealed crowding of hair follicles. There were no other skin lesions and no ophthalmic abnormalities. Ictal EEG showed a theta burst in the right parietal area. Computed tomography of the brain revealed cerebral atrophy and a slightly high intensity lesion in the right parietal and occipital lobes with calcification. Gyriform enhancement was demonstrated by contrast MRI in these areas. The pathogenesis is discussed. This case may represent a previously unknown neurocutaneous syndrome.