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      Cherubism with idiopathic gingival enlargement: A rare case report

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          Abstract

          Cherubism is a congenital childhood disease of autosomal dominant inheritance. It is a benign, familial giant cell lesion characterized by gradually progressive painless swelling of the jaws. Idiopathic gingival enlargement is a rare condition and may be associated with some uncommon syndromes. This case report describes an 11-year-old patient with unusual clinical form of gingival enlargement, cherubic facial appearance. Clinical examination revealed the presence of the hyperplastic gingiva, which completely covered all teeth. The bilateral swelling of mandible and the appearance of the sclera beneath the iris suggested cherubism. The diagnosis was confirmed by histopathological examination, which revealed multinucleated giant cells. Computed tomography scan showed multiple osteolytic zones in the mandible. A full mouth gingivectomy was performed in four stages. Lesion healed successfully, and no recurrence observed after 1-year follow-up. There was a marked improvement in esthetics and function through the surgical excision of the overgrowth.

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          Most cited references13

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          Clinicopathologic study of 24 cases of cherubism.

          The authors reviewed 24 cases of familial or nonfamilial cherubism. The age at onset was between 6 and 10 years. It was characterized by bilateral painless swelling of jaws and eyes-to-heaven appearance was visible when the maxillae were affected as well. Radiographs showed well-defined multilocular radiolucencies and with age, thick sclerotic borders were visible. A malocclusive and abnormal dentition, worse in the mandible can be seen. Histopathologically, numerous randomly distributed multinucleated giant cells and vascular spaces within a fibrous connective tissue stroma with or without eosinophilic collagen perivascular cuffing were apparent. Multinucleated giant cells were positive for osteoclastic specific markers, tartrate-resistant acid phosphatase and human alphaV beta3 integrin, 23C6. Results after follow-up were available for 14 cases. Of these, no treatment was carried out in five cases, cherubism resolved (three cases) or grew slowly (two cases); curettage or surgical contouring was performed in seven cases, during the rapid growth of the lesions. This not only gave good immediate results, but also arrested active growth of remnant cherubic lesions and even stimulated bone regeneration. Segmental mandibulectomy followed by reconstruction was performed in two cases with extensive lesion and the risk of pathologic fracture of the mandible, and excellent results were obtained.
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            Familial gingival fibromatosis with unusual histologic findings.

            This article describes the occurrence of numerous calcifications, amyloid deposits, and islands of odontogenic epithelium in the gingiva of 3 siblings with familial gingival fibromatosis. These microscopic features have not been reported previously in patients with this condition.
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              Cherubism and its charlatans.

              Cherubism is a rare hereditary condition characterised by progressive cystic proliferation of the mandible and maxilla in childhood, followed by post-pubertal involution of the process and jaw remodelling in adulthood. Its name is derived from the cherubic appearance that results from the jaw hypertrophy. Here, we present the case of a young boy with cherubism, in the context of his pedigree, to illustrate the clinical characteristics and their variable expression. We also seek to distinguish cherubism from central giant cell granuloma and giant cell tumour of the jaws, with which it holds a false synonymity. Copyright 2001 The British Association of Plastic Surgeons.
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                Author and article information

                Journal
                J Indian Soc Periodontol
                J Indian Soc Periodontol
                JISP
                Journal of Indian Society of Periodontology
                Medknow Publications & Media Pvt Ltd (India )
                0972-124X
                0975-1580
                Nov-Dec 2017
                : 21
                : 6
                : 503-506
                Affiliations
                [1] Department of Periodontics, JKK Nattaraja Dental College, Komarapalayam, Tamil Nadu, India
                [1 ] Department of Periodontia, Azeezia Dental College, Meyanoor, Kollam, Kerala, India
                Author notes
                Address for correspondence: Dr. Sugumari Elavarasu, 137, Sambu Enclave, Salem Main Road, Komarapalayam, Tamil Nadu, India. E-mail: drsugu@ 123456gmail.com
                Article
                JISP-21-503
                10.4103/jisp.jisp_318_13
                5846250
                d2f78374-b335-4ca1-9989-7986bfdbb7eb
                Copyright: © 2018 Indian Society of Periodontology

                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.

                History
                : 06 November 2013
                : 09 March 2015
                Categories
                Case Report

                Dentistry
                cherubism,idiopathic gingival enlargement,gingivectomy
                Dentistry
                cherubism, idiopathic gingival enlargement, gingivectomy

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