Cherubism

Cherubism is an uncommon, benign fibro-osseous lesion which causes a progressive, painless, symmetrical expansion of the jaws. It is primarily found in the mandible. The disease was first described in 1933 by Jones [1], who called it familial multilocular disease of the jaws. He coined the term cherubism, because the affected persons resembled the figure in Renaissance art that had a round full-face appearance and upturned eyes. Peter reported a family with no less than 20 affected members. Zohar et al. were able to trace, in an unbroken line through four generations, family members who showed either fibrous dysplasia or cherubism. According to the WHO classification, cherubism belongs to a group of nonneoplastic bone lesions affecting only the jaws. It is a rare, benign condition with autosomal dominant inheritance, and it is one of the very few genetically determined osteoclastic lesions in the human body. It appears to have 100% penetrance in males and only 50–70% penetrance in females with 2:1 male predominance.