The congenital neurocutaneous syndromes are a group of related disorders characterized by dysplastic abnormalities of the skin and nervous system.1 Included in this diagnostic category are tuberous sclerosis, neurofibromatosis, Sturge-Weber syndrome, and ataxia telangiectasia.2-5 In all of these, characteristic skin lesions, usually of the face, are associated with functional and anatomic evidence of central nervous system lesions. It is the consensus that these lesions, both dermal and neural, are dysplastic rather than neoplastic and are of a congenital nature whether manifested at birth or not until some later time.
Recently we had the opportunity of studying 2 patients with unusual linear nevi of the face associated with convulsions and mental retardation. The striking similarity of the clinical course and gross and microscopic identity of the skin lesions suggests that this might represent a distinct entity perhaps related to the neurocutaneous syndromes.
Report of Cases
Case 1.—The patient was
FEUERSTEIN RC, MIMS LC. Linear Nevus Sebaceus with Convulsions and Mental Retardation. Am J Dis Child. 1962;104(6):675-679. doi:10.1001/archpedi.1962.02080030675013