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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...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Medicina Oral S.L.
2014
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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. |
format | Online Article Text |
id | pubmed-4134864 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Medicina Oral S.L. |
record_format | MEDLINE/PubMed |
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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