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CASE REPORT
Year : 2012  |  Volume : 3  |  Issue : 4  |  Page : 514-518

Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)


Department of Pedodontics and Preventive Dentistry, Sri Siddhartha Dental College and Hospital, Agalakote, Tumkur, Karnataka, India

Correspondence Address:
N K Kiran
Department of Pedodontics and Preventive Dentistry, Sri Siddhartha Dental College and Hospital, Agalakote, Tumkur 502 107, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0976-237X.107459

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The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.


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