Oral and Dental Findings of Dyskeratosis Congenita

Dyskeratosis congenital (DC) is a rare condition characterized by reticulate skin hyperpigmentation, mucosal leukoplakia, and nail dystrophy. More serious features are bone marrow involvement with pancytopenia and a predisposition to malignancy. The purpose of this case report is to describe the ora...

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Main Authors: Mine Koruyucu, Pelin Barlak, Figen Seymen
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Dentistry
Online Access:http://dx.doi.org/10.1155/2014/454128
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author Mine Koruyucu
Pelin Barlak
Figen Seymen
author_facet Mine Koruyucu
Pelin Barlak
Figen Seymen
author_sort Mine Koruyucu
collection DOAJ
description Dyskeratosis congenital (DC) is a rare condition characterized by reticulate skin hyperpigmentation, mucosal leukoplakia, and nail dystrophy. More serious features are bone marrow involvement with pancytopenia and a predisposition to malignancy. The purpose of this case report is to describe the oral and dental findings in children with DC syndrome. A 10-year-old male diagnosed with DC was admitted because of extensive caries and toothache. Inadequate oral hygiene and extensive caries were observed in oral examination of the patient. Plaque accumulation was seen in gingival border of maxillary teeth. Papillary atrophy on the tongue was observed. Short and blunted roots of mandible incisors and upper and lower molars were determined on the radiographic examination. Dryness on the lips and commisuras, ectropion on his eyes, and epiphora were observed. Hematologic tests were performed and showed aplastic anemia at the age of 2. At the age of 4, the bone marrow transplantation was performed. Dermatological findings occurred after the bone marrow transplantation. The skin of the patient was thin, dry, and wrinkled in some areas. He had palmoplantar hyperkeratosis and syndactylia on his fingers. Endodontic treatment procedures were applied and other extensive caries are still being restored. The patient will be given full preventive care during regular follow-up. Oral hygiene was improved to the optimum level.
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spelling doaj-art-ae94a2a3cab74873994853ddc1972bb32025-02-03T06:46:23ZengWileyCase Reports in Dentistry2090-64472090-64552014-01-01201410.1155/2014/454128454128Oral and Dental Findings of Dyskeratosis CongenitaMine Koruyucu0Pelin Barlak1Figen Seymen2Faculty of Dentistry, Department of Pedodontics, Istanbul University, 34093 Istanbul, TurkeyFaculty of Dentistry, Department of Pedodontics, Istanbul University, 34093 Istanbul, TurkeyFaculty of Dentistry, Department of Pedodontics, Istanbul University, 34093 Istanbul, TurkeyDyskeratosis congenital (DC) is a rare condition characterized by reticulate skin hyperpigmentation, mucosal leukoplakia, and nail dystrophy. More serious features are bone marrow involvement with pancytopenia and a predisposition to malignancy. The purpose of this case report is to describe the oral and dental findings in children with DC syndrome. A 10-year-old male diagnosed with DC was admitted because of extensive caries and toothache. Inadequate oral hygiene and extensive caries were observed in oral examination of the patient. Plaque accumulation was seen in gingival border of maxillary teeth. Papillary atrophy on the tongue was observed. Short and blunted roots of mandible incisors and upper and lower molars were determined on the radiographic examination. Dryness on the lips and commisuras, ectropion on his eyes, and epiphora were observed. Hematologic tests were performed and showed aplastic anemia at the age of 2. At the age of 4, the bone marrow transplantation was performed. Dermatological findings occurred after the bone marrow transplantation. The skin of the patient was thin, dry, and wrinkled in some areas. He had palmoplantar hyperkeratosis and syndactylia on his fingers. Endodontic treatment procedures were applied and other extensive caries are still being restored. The patient will be given full preventive care during regular follow-up. Oral hygiene was improved to the optimum level.http://dx.doi.org/10.1155/2014/454128
spellingShingle Mine Koruyucu
Pelin Barlak
Figen Seymen
Oral and Dental Findings of Dyskeratosis Congenita
Case Reports in Dentistry
title Oral and Dental Findings of Dyskeratosis Congenita
title_full Oral and Dental Findings of Dyskeratosis Congenita
title_fullStr Oral and Dental Findings of Dyskeratosis Congenita
title_full_unstemmed Oral and Dental Findings of Dyskeratosis Congenita
title_short Oral and Dental Findings of Dyskeratosis Congenita
title_sort oral and dental findings of dyskeratosis congenita
url http://dx.doi.org/10.1155/2014/454128
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AT figenseymen oralanddentalfindingsofdyskeratosiscongenita