Abstract
Cherubism is an autosomal dominant, very rare benign fibro-osseous lesion, with an estimation of only 300 cases have been reported worldwide. Cherubism is characterized clinically by bilateral symmetrical self-limiting jaw enlargement starts in the childhood or early teens.Mandible is mostly affected although maxilla and zygomatic bones may be involved but to a lower extent), and is associated with tooth displacement and severe malocclusion. Histopathologically, it is indistinguishable from giant cell lesions therefore the clinical manifestations of the disease remain the Gold Stone. Keywords: Cherubism, mandible, maxilla, bilateral palatal swelling
Highlights
Cherubism is a rare, non –neoplastic, selflimiting, hereditary fibro-osseous disorder of the jaws [1]
The disease is inherited and the type of inheritance is autosomal dominant through mutation of SH3-binding protien2(SH3bp2) gene, leading to the formation of bilateral multilocular radiolucent cystic lesions containing fibrous tissue leading to bilateral enlargement of the affected jaw, which is in the most cases the
The resulting fullness of the face may lead to the swelling of the submandibular lymph nodes, and the expanded masses of the dysplastic fibrous soft tissue can infiltrate the floor of the orbit, leading to the upward movement of the eyeball showing up the sclera under the iris giving the feature of upward gaze[2]
Summary
Non –neoplastic, selflimiting, hereditary fibro-osseous disorder of the jaws [1]. It had been defined as a different kind of the other multilocular cystic diseases of the jaws by William A. Due to the disease rarity, only 300 cases of Cherubism had been described to date [3]. The disease is inherited and the type of inheritance is autosomal dominant through mutation of SH3-binding protien2(SH3bp2) gene, leading to the formation of bilateral multilocular radiolucent cystic lesions containing fibrous tissue leading to bilateral enlargement of the affected jaw, which is in the most cases the. 2021 Diyala Journal of Medicine mandible, sometimes both the mandible and the maxillae are both affected [3]
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