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Nonfamilial cherubism: A case report and review of literature
Cherubism is a rare hereditary developmental condition of the jaws and generally inherited as an autosomal dominant trait. It is also known as familial fibrous dysplasia of the jaws, familial multilocular cystic disease and hereditary fibrous dysplasia of the jaws. The gene for cherubism is mapped t...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Medknow Publications & Media Pvt Ltd
2017
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5406808/ https://www.ncbi.nlm.nih.gov/pubmed/28479714 http://dx.doi.org/10.4103/0973-029X.203791 |
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author | Deshmukh, Revati Joshi, Samir Deo, Priya Nimish |
author_facet | Deshmukh, Revati Joshi, Samir Deo, Priya Nimish |
author_sort | Deshmukh, Revati |
collection | PubMed |
description | Cherubism is a rare hereditary developmental condition of the jaws and generally inherited as an autosomal dominant trait. It is also known as familial fibrous dysplasia of the jaws, familial multilocular cystic disease and hereditary fibrous dysplasia of the jaws. The gene for cherubism is mapped to chromosome 4p16.3 may lead to pathologic activation of osteoclasts and disruption of jaw morphogenesis. The lesion usually appears between 2 and 5 years shows a predilection for the mandible and causes a bilateral swelling giving rise to a cherubic chubby appearance. The eosinophilic cuffing of blood vessels appears to be specific for cherubism. The diagnosis is based on clinical, radiographic and histopathologic findings. The purpose of this article is to present a rare case of nonfamilial cherubism as there are very few cases reported and to review the literature with its cone beam computed tomography findings. |
format | Online Article Text |
id | pubmed-5406808 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Medknow Publications & Media Pvt Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-54068082017-05-05 Nonfamilial cherubism: A case report and review of literature Deshmukh, Revati Joshi, Samir Deo, Priya Nimish J Oral Maxillofac Pathol Case Report Cherubism is a rare hereditary developmental condition of the jaws and generally inherited as an autosomal dominant trait. It is also known as familial fibrous dysplasia of the jaws, familial multilocular cystic disease and hereditary fibrous dysplasia of the jaws. The gene for cherubism is mapped to chromosome 4p16.3 may lead to pathologic activation of osteoclasts and disruption of jaw morphogenesis. The lesion usually appears between 2 and 5 years shows a predilection for the mandible and causes a bilateral swelling giving rise to a cherubic chubby appearance. The eosinophilic cuffing of blood vessels appears to be specific for cherubism. The diagnosis is based on clinical, radiographic and histopathologic findings. The purpose of this article is to present a rare case of nonfamilial cherubism as there are very few cases reported and to review the literature with its cone beam computed tomography findings. Medknow Publications & Media Pvt Ltd 2017 /pmc/articles/PMC5406808/ /pubmed/28479714 http://dx.doi.org/10.4103/0973-029X.203791 Text en Copyright: © 2017 Journal of Oral and Maxillofacial Pathology http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Deshmukh, Revati Joshi, Samir Deo, Priya Nimish Nonfamilial cherubism: A case report and review of literature |
title | Nonfamilial cherubism: A case report and review of literature |
title_full | Nonfamilial cherubism: A case report and review of literature |
title_fullStr | Nonfamilial cherubism: A case report and review of literature |
title_full_unstemmed | Nonfamilial cherubism: A case report and review of literature |
title_short | Nonfamilial cherubism: A case report and review of literature |
title_sort | nonfamilial cherubism: a case report and review of literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5406808/ https://www.ncbi.nlm.nih.gov/pubmed/28479714 http://dx.doi.org/10.4103/0973-029X.203791 |
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