[Encephalocraniocutaneous lipomatosis].
BACKGROUND
Encephalocraniocutaneous lipomatosis (ECL) involving the scalp and cerebellum was observed without asymptomatic expression.


METHODS
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.


CONCLUSIONS
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.
Louvet
S
S
Unité de Dermatologie Pédiatrique, Hôpital Pellegrin-Enfants Bordeaux.
Léauté-Labreze
C
C
Labbé
L
L
Chateil
J F
JF
Taieb
A
A
D001927:Brain Diseases / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007223:Infant; D008068:Lipomatosis / Q000150:complications*; D008279:Magnetic Resonance Imaging; D008297:Male; D011859:Radiography; D012536:Scalp Dermatoses / Q000209:etiology*; D012886:Skull / Q000002:abnormalities / Q000000981:diagnostic imaging