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Cherubism combined with epilepsy, mental retardation and gingival fibromatosis (Ramon syndrome): a case report
J Suhanya1, Chakshu Aggarwal, Khadijah Mohideen
1Department of Oral Medicine and Radiology, Tamil Nadu Government Dental College and Hospital, Chennai, 600 003, India.
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.
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