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Imaging Sci Dent. 2012 Mar;42(1):55-60. doi: 10.5624/isd.2012.42.1.55. Epub 2012 Mar 22.

Radiological features of familial Gorlin-Goltz syndrome.

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1
Department of Oral Medicine and Radiology, AB Shetty Memorial Institute of Dental Sciences, Nitte University, Mangalore, India.

Abstract

Gorlin-Goltz syndrome is an autosomal dominant disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocystic odontogenic tumors, and skeletal anomalies. This syndrome may be diagnosed early by dentist because keratocystic odontogenic tumors are usually one of the first manifestations of the syndrome. Early diagnosis and treatment are of utmost importance in reducing the severity of long term sequelae of this syndrome. This report presents a rare event of Gorlin-Goltz syndrome occurring in a 39-year-old male and his 8-year-old daughter. The clinical and investigative features of this familial disorder has been described in detail.

KEYWORDS:

Basal Cell Nevus Syndrome; Odontogenic Cysts; Skeletal Anomalies

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