Sturge-Weber Syndrome

PHAKOMATOSES¹is the term that has been applied to the neurocutaneous syndromes. This term is derived from the Greek root [unk], which means "mother spot" or "mole" and implies a congenital factor. The three diseases which comprise this group and which have cutaneous lesions are (1) tuberous sclerosis, (2) neurofibromatosis of Von Recklinghausen and (3) Sturge-Weber disease. The first two are familiar to the dermatologist. However, there has been a paucity of dermatologic writing on the Sturge-Weber syndrome. In the American dermatologic literature cases have been reported by Goldberg,²who cited an instance of nevus flammeus (hemangioma congenitale) associated with glaucoma. Butterworth³reported the case of an epileptic idiot, a woman aged 20, with angiomatosis of the brain and a vascular nevus of the trigeminal area of the skin. Gammel⁴reported the case of a boy with a port wine nevus of the face associated with