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Cherubism: report of a rare pathology

Author: 
Sugunakar Raju
Subject Area: 
Health Sciences
Abstract: 

Cherubism is a skeletal dysplasia characterized by bilaterally symmetric fibro-osseous lesions limited to the mandible and maxilla. Affected children appear normal at birth. Swelling of the jaws usually appears between 2 and 7 years of age, after which, lesions proliferate and increase in size until puberty. The lesions subsequently begin to regress, fill with bone and remodel until age 30, when they are frequently not detectable. Cherubism is due to autosomal dominant mutations in the SH3BP2 gene on chromosome 4p16.3. The lesions seen in Cherubism are painless and more or less symmetrical. Frequently, Cherubism is accompanied by dental arch and tooth eruption abnormalities with rare extra gnathic skeletal involvement. The rounded face, due to jaw hypertrophy and upward gaze with exposure of the sclera below the pupil gives a reminder of cherubs depicted in Renaissance paintings. This paper reports a case of non-familial cherubism with its clinical, radiological, histopathological features along with treatment aspects.

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