ENCEPHALOCRANIOCUTANEOUS LIPOMATOSIS

Background. Encephalocranicutaneous lipomatosis (ECL) involving the sc alp and cerebellum was observed without asymptomatic expression. Case report. A seven-month-old infant presented with two soft subcutaneous hairless tumors of the scalp without any associated clinical anomaly. Neuroradiology explorations (radiography, CT-scan and MRI) showed a li poma in the cerebellum linked with a occipital cutaneous lipoma throug h a bone defect. At the age of 3 years, the child remains healthy. Dis cussion. ECL is a rare neurocutaneous disorder that consists of skin l ipomas associated with various cerebral anomalies. ECL may occur as a circumscribed form of the Proteus syndrome, since a few ECL patients h ave associated manifestations of Proteus syndrome as well as localized hypertrophy. However, minor forms of ECL are possible and may be comp atible with normal life.