[Encephalocraniocutaneous lipomatosis].

BACKGROUND

Encephalocraniocutaneous lipomatosis (ECL) involving the scalp 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 lipoma in the cerebellum linked with a occipital cutaneous lipoma through a bone defect. At the age of 3 years, the child remains healthy.

DISCUSSION: ECL is a rare neurocutaneous disorder that consists of skin lipomas associated with various cerebral anomalies. ECL may occur as a circumscribed form of the Proteus syndrome, since a few ECL patients have associated manifestations of proteus syndrome as well as localized hypertrophy. However, minor forms of ECL are possible and may be compatible with normal life.