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Aggressive behaviour of cherubism in a teenager: 4-years of clinical follow-up associated with radiographic and histological features

¹ Department of Biosciences and Oral Diagnosis, São José dos Campos Dental School, São Paulo State University, UNESP, and Chairman of Bioscience Center for Special Health Care Needs and Special Health Care Needs Association, São José dos Campos, São Paulo, Brazil; ² São José dos Campos Dental School, São Paulo State University, UNESP, São Paulo, Brazil; ³ Department of Radiology, São José dos Campos Dental School, São Paulo State University, UNESP, São José dos Campos, São Paulo, Brazil; ⁴ Private Practice in Oral and Maxillofacial Surgery and Prosthesis, Taubaté, São Paulo, Brazil; ⁵ Department of Biosciences and Oral Diagnosis, São José dos Campos School of Dentistry, São Paulo State University, UNESP, São José dos Campos, São Paulo, Brazil

*Correspondence to: Dr Mônica Fernandes Gomes, Faculdade de Odontologia de São José dos Campos, UNESP, Departamento de Biociências e Diagnóstico Oral, Av. Eng. Francisco José Longo, 777 / Cep: 12.245-000, São José dos Campos / São Paulo / Brasil; E-mail: mfgomes{at}fosjc.unesp.br

Received 7 October 2004; revised 3 February 2005; accepted 6 March 2005

Cherubism is a rare hereditary fibro-osseous childhood disease characterized by bone degradation and fibrous tissue replacement at the angles of the mandible and at the tuberosity areas of the maxilla that leads to prominence of the lower face and an appearance reminiscent of the cherub's portrayal in Renaissance art. This disease has an autosomal dominant hereditary characteristic. The purpose of this report is to analyse laboratory tests, clinicopathological and radiographic features of cherubism and its intraoral manifestations in a patient during 4-years of follow-up, correlating the features observed in this case with those of the literature. Also discussed is the atypical and aggressive behaviour of this case during puberty.

Keywords: cherubism; maxillofacial; jaw complex; intraoral manifestations