Familial multilocular cystic disease of the jaws

 # Which of the following is also known as familial multilocular cystic disease of the jaws?

A. Osteopetrosis

B. Cleidocranial dysostosis

C. Hemifacial microsomia

D. Cherubism

The correct answer is D. Cherubism.

Cherubism, a non-neoplastic hereditary bone lesion that is histologically similar to central giant cell granuloma, affects the jaws of children bilaterally and symmetrically, usually producing the so-called cherubic look (Fig. 17-13). The disease was first described in 1933 by Jones, who called it familial multilocular disease of the jaws. The term ‘cherubism’, was introduced by Jones and others to describe the clinical appearance of affected patients. According to the WHO classification, cherubism belongs to a group of non-neoplastic 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. There is great variation in the clinical expression.

Reference: Shafer’s Textbook of ORAL PATHOLOGY, 7th Edition, Page No: 715

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