[Encephalocraniocutaneous lipomatosis and linear naevus sebaceus]

Abstract

Encephalocraniocutaneous lipomatosis is a rare neurocutaneous dysgenetic syndrome, which is characterized by unilateral lipomas in the subcutis of the face, skalp and skull, porencephalic cyst of the ipsilateral hemisphere, epilepsy, and severely delayed development. Furthermore a variety of skin lesions (choristomas) has been described. In the present case the skin lesions had the form of a linear nevus sebaceus. This condition is frequently combined with epilepsy and neurological disorders (Schimmelpenning-Feuerstein-Mims-syndrome). The latter seems not to be a very rare syndrome. The coincidence of characteristics of both syndromes in the same child supports the suggestion of a pathogenetical relationship.