Hemamalini Chandrashekhar¹*, Pallavi Ranadive² and Mohan Deshpande³

¹Oral and Maxillofacial Surgeon, Dental Solutions, Former Post Graduate Resident, Department of Oral and Maxillofacial Surgery, Nair Hospital Dental College, Mumbai, India
²Associate Professor (Additional), Department of Oral and Maxillofacial Surgery, Nair Hospital Dental College, Mumbai, India
³Professor, Department of Oral and Maxillofacial Surgery, Nair Hospital Dental College, Mumbai, India

*Corresponding Author: Hemamalini Chandrashekhar, Oral and Maxillofacial Surgeon, Dental Solutions, Former Post Graduate Resident, Department of Oral and Maxillofacial Surgery, Nair Hospital Dental College, Mumbai, India.

Received: October 28, 2021; Published:00-00

Abstract

Dyskeratosis congenita (DC) is a rare telomere disorder presenting with distinct clinical and genetic heterogeneity. The classical DC comprises of a triad- skin pigmentation, nail dystrophy and oral leucoplakia. However, DC can manifest with a variety of multi-organ involvement, mainly the haemopoetic, respiratory and gastrointestinal. Predisposition to cancers and bone marrow failure are often the reasons for early mortality. Oral findings of DC include rampant dental caries, periodontal disease and taurodontism.

In this paper, we present a rare case report of Dyskeratosis Congenita in two siblings of opposite gender. This case report mainly aims to address the challenges in diagnosing this rare condition, by revisiting its clinical features, particularly the oral manifestations. It is also intended to supplement data of DC in females, in whom the condition is extremely rare.

Keywords: Dyskeratosis Congenita; Pre-cancerous Condition; Telomere Disorder; Zinsser-Cole-Engman Syndrome

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Citation

Citation: Chandrashekhar H., et al. “Dyskeratosis Congenita in Siblings: A Rare Case Report". Acta Scientific Dental Sciences 5.12 (2021):.

Copyright

Copyright: © 2021 Chandrashekhar H., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.