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Case Reports in Dentistry
Volume 2012 (2012), Article ID 475439, 4 pages
http://dx.doi.org/10.1155/2012/475439
Case Report

Gorlin-Goltz Syndrome: Case Report of a Rare Hereditary Disorder

1Department of Oral Pathology and Microbiology, Institute of Dental Sciences, Uttar Pradesh, Bareilly 243006, India
2Department of Oral Pathology and Microbiology, Padmashree Dr. D. Y. Patil Dental College and Hospital, Nerul, Maharashtra, Navi Mumbai 400706, India
3Department of Oral Pathology and Microbiology, Kothiwal Dental College and Research Centre, Uttar Pradesh, Moradabad 244001, India

Received 4 July 2012; Accepted 28 August 2012

Academic Editors: J. N. dos Santos and M. A. Polack

Copyright © 2012 Ashutosh Agrawal et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Introduction. Gorlin-Goltz syndrome is an inherited autosomal dominant disorder with complete penetrance and extreme variable expressivity. Case Report. The present paper highlights the importance of diagnostic criteria and histopathology in early and prompt diagnosis which will lead to proper treatment and genetic counseling of the patient. Discussion. Gorlin-Goltz syndrome is about multisystem process comprising the triad of basal cell nevi, jaw keratocysts, and skeletal anomalies. A spectrum of other neurological, ophthalmic, endocrine and genital manifestations is known to be variably associated with this triad. Diagnosis of the syndrome is based on major and minor criteria. Conclusion. This paper emphasizes the importance of oral and maxillofacial health professionals in the early diagnosis of nevoid basal cell carcinoma syndrome and in a preventive multidisciplinary approach to provide a better prognosis to the patient.