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Open AccessFocal dermal hypoplasia: oral and dental findings
Focal dermal hypoplasia: oral and dental findings
1Maxillofacial and Dental Department, Great Ormond Street Hospital for Children NHS Trust
DOI: 10.17796/jcpd.30.1.q737147154231251 Vol.30,Issue 1,January 2006 pp.67-72
Published: 01 January 2006
*Corresponding Author(s): Zahra Tejani E-mail: Zahrat@gmail.com
Abstract
Focal Dermal Hypoplasia/Goltz Gorlin syndrome is a rare syndrome characterized by widespread dysplasia affecting tissues of mesodermal and ectodermal origin with cutaneous, osseous, ocular, oral and dental defects. Enamel hypoplasia is the most commonly reported dental manifestation and has recently been described as a possible manifestation of Lyonisation. This article reviews the reported dental findings and reports a new case with typical findings of focal dermal hypoplasia, which has been under review on our department for 10 years. It discusses the differential diagnosis as well as newer concepts of aetiology and pathogenesis in relation to dental anomalies.
Enamel hypoplasia may make plaque control difficult, resulting in generalized gingivitis. Hand anomalies may limit dexterity and exacerbate this. From the dental standpoint we emphasize the implementation of timely preventive and/or therapeutic strategies. Since there are periods of exacerba-tion during the course of this syndrome, regular surveillance from an early age with the frequency of visits increasing during and after adolescence is indicated. The role of the dentist in improving aesthetics and function can have tremendous psychological impact to enhance self-esteem of such patients.
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Zahra Tejani,Puneet Batra,Carol Mason,David Atherton. Focal dermal hypoplasia: oral and dental findings. Journal of Clinical Pediatric Dentistry. 2006. 30(1);67-72.
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