Search

Three infants had linear sebaceous nevus syndrome. It is suggested that this syndrome be considered a hamartoma variant. In the first patient, postmortem examination of the kidneys revealed bilateral nodular nephroblastomatosis. Cortical blindness and intractable seizures were noted in the second patient. Cardiac involvement was present in two patients. Similarities between linear sebaceous nevus syndrome and tuberous sclerosis include involvement of numerous organ systems in addition to the skin and brain.

Maternal phenylketonuria is likely to have damaging effects on the offspring. Intrauterine growth retardation, postnatal growth retardation, microcephaly, mental retardation, congenital malformations in the infant, and spontaneous abortions in the mother suggest the possibility of maternal phenylketonuria even if the mother is of normal intelligence.

While hospitalized, a 3-year-old girl went into coma on four separate occasions. Neurologic evaluation of this patient suggested a toxic encephalopathy. Following exhaustive pharmacologic investigation, toxic quantities of chloral hydrate were isolated from the child's blood, urine, and gastric contents. It was discovered that the child's mother had tried to poison her daughter with the drug. This unusual case demonstrates the value of perseverance in the clarification of an ill-defined clinical problem.

We attempted to clarify existing nomenclature utilized in describing linear epidermal nevi and Dr. Lantis' letter illustrates the lack of uniformity in classification of the dermatological entities. The references quoted by Dr. Lantis were reviewed by us and only because of space limitations, were not included in the reference section. Biopsies were performed in all three cases and histologic findings of hyperkeratosis and parakeratosis were common to all.We wish to emphasize that the linear sebaceous nevus syndrome is representative of one of several types of linear epidermal nevi. Classification of neurocutaneous syndromes into groups and subgroups is far less important than attempting to delineate the pathogenesis of these entities. Furthermore, we do not believe that the nomenclature referred to in the article will mislead or create confusion, particularly among pediatricians. Dr. Lantis appears to be engaged in a battle of semantics and has completely overlooked the authors' intent to