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Zinsser-Cole-Engmann syndrome: A rare case report with literature review

Zinsser-Cole-Engmann syndrome, more commonly known as Dyskeratosis Congenita, is a heritable genodermatosis having an estimated incidence of 1 in 1 million people. It is important for an oral physician to be aware of this condition as oral leukoplakia occurs in this condition as part of a classic tr...

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Autores principales: Chalkoo, Altaf H., Kaul, Vibhuti, Wani, Lateef A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medicina Oral S.L. 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4134864/
https://www.ncbi.nlm.nih.gov/pubmed/25136436
http://dx.doi.org/10.4317/jced.51274
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author Chalkoo, Altaf H.
Kaul, Vibhuti
Wani, Lateef A.
author_facet Chalkoo, Altaf H.
Kaul, Vibhuti
Wani, Lateef A.
author_sort Chalkoo, Altaf H.
collection PubMed
description Zinsser-Cole-Engmann syndrome, more commonly known as Dyskeratosis Congenita, is a heritable genodermatosis having an estimated incidence of 1 in 1 million people. It is important for an oral physician to be aware of this condition as oral leukoplakia occurs in this condition as part of a classic triad along with reticulate skin pigmentation and nail dystrophy. Besides these, there may be myriad multisystem involvement as well. These individuals have a high predilection for developing malignancies as well as other grave life-threatening conditions. Timely diagnosis and management of these cases may help improve their morbidity and mortality, for which oral physicians can play a major role in recognizing the cases. This will only be possible when more of such cases are reported in dental literature. Here we present a case report of a 30 year old male patient who reported to our department with all the characteristic features of the triad and a few additional findings concordant to the disease as well. Key words:Zinsser-Cole-Engmann syndrome, Dyskeratosis Congenita, leukoplakia, genodermatosis, skin pigmentation, nail dystrophy, progeria, hematological disturbances.
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spelling pubmed-41348642014-08-18 Zinsser-Cole-Engmann syndrome: A rare case report with literature review Chalkoo, Altaf H. Kaul, Vibhuti Wani, Lateef A. J Clin Exp Dent Case Report Zinsser-Cole-Engmann syndrome, more commonly known as Dyskeratosis Congenita, is a heritable genodermatosis having an estimated incidence of 1 in 1 million people. It is important for an oral physician to be aware of this condition as oral leukoplakia occurs in this condition as part of a classic triad along with reticulate skin pigmentation and nail dystrophy. Besides these, there may be myriad multisystem involvement as well. These individuals have a high predilection for developing malignancies as well as other grave life-threatening conditions. Timely diagnosis and management of these cases may help improve their morbidity and mortality, for which oral physicians can play a major role in recognizing the cases. This will only be possible when more of such cases are reported in dental literature. Here we present a case report of a 30 year old male patient who reported to our department with all the characteristic features of the triad and a few additional findings concordant to the disease as well. Key words:Zinsser-Cole-Engmann syndrome, Dyskeratosis Congenita, leukoplakia, genodermatosis, skin pigmentation, nail dystrophy, progeria, hematological disturbances. Medicina Oral S.L. 2014-07-01 /pmc/articles/PMC4134864/ /pubmed/25136436 http://dx.doi.org/10.4317/jced.51274 Text en Copyright: © 2014 Medicina Oral S.L. http://creativecommons.org/licenses/by/2.5/ 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 work is properly cited.
spellingShingle Case Report
Chalkoo, Altaf H.
Kaul, Vibhuti
Wani, Lateef A.
Zinsser-Cole-Engmann syndrome: A rare case report with literature review
title Zinsser-Cole-Engmann syndrome: A rare case report with literature review
title_full Zinsser-Cole-Engmann syndrome: A rare case report with literature review
title_fullStr Zinsser-Cole-Engmann syndrome: A rare case report with literature review
title_full_unstemmed Zinsser-Cole-Engmann syndrome: A rare case report with literature review
title_short Zinsser-Cole-Engmann syndrome: A rare case report with literature review
title_sort zinsser-cole-engmann syndrome: a rare case report with literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4134864/
https://www.ncbi.nlm.nih.gov/pubmed/25136436
http://dx.doi.org/10.4317/jced.51274
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