Cherubism combined with epilepsy, mental retardation and gingival fibromatosis (Ramon syndrome): a case report.

ABSTRACT: Cherubism is an inherited, autosomal dominant disorder that characteristically affects the jaws of children. The disease typically manifest as a bilateral swelling with associated submandibular lymph node enlargements and usually regresses as age advances. The disease is microscopically indistinguishable from other giant cell lesions and is essentially a clinical diagnosis. The association of cherubism with gingival fibromatosis, epilepsy, mental retardation, stunted growth, and hypertrichosis is referred as Ramon syndrome. We report a case of Ramon syndrome in an 8 year old girl.

SUBMITTER: Suhanya J 

PROVIDER: S-EPMC2878617 | biostudies-literature | 2010 Jun

REPOSITORIES: biostudies-literature