Baso-cellular Naevomatose

The baso-cellular naevomatose is a Genetic disease appearing by small tumefactions of the skin: Kératokyste S of the Jaw S starting between 10 and 20 years or of the baso-cellular naevi starting between 20 and 30 years.

Nearly 60% of the individuals have an aspect of joint face: macrocephaly, projecting face, coarse face and milium of the face. The majority of the patients are carrying coasts bifides or anomalies of the vertebrae. Calcifications of the forgery of the brain is found in more than 90% of the cases after 20 years.

Cardiac Fibrome S or ovariens meet respectively in 2 to 20% of the patients. 5% of the children will develop a Médulloblastome with a maximum with age the 2 years. The life expectancy of these patients is not decreased.

Sources

• D Gareth Evans, Peter has Farndon, Nevoid Basal Concealment Carcinoma Syndrome In GeneTests: Medical Genetics Information Resource (database online). Copyright, University off Washington, Seattle. 1993-2005

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