Published in:
01-06-2010 | Case Report
Cherubism Combined with Epilepsy, Mental Retardation and Gingival Fibromatosis (Ramon Syndrome): A Case Report
Authors:
J. Suhanya, Chakshu Aggarwal, Khadijah Mohideen, S. Jayachandran, I. Ponniah
Published in:
Head and Neck Pathology
|
Issue 2/2010
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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.