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Zinsser-Cole-Engmann syndrome: A rare case report with literature review.
MedLine Citation:
PMID:  25136436     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Altaf H Chalkoo; Vibhuti Kaul; Lateef A Wani
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Publication Detail:
Type:  Journal Article     Date:  2014-07-01
Journal Detail:
Title:  Journal of clinical and experimental dentistry     Volume:  6     ISSN:  1989-5488     ISO Abbreviation:  J Clin Exp Dent     Publication Date:  2014 Jul 
Date Detail:
Created Date:  2014-08-19     Completed Date:  2014-08-19     Revised Date:  2014-08-21    
Medline Journal Info:
Nlm Unique ID:  101603132     Medline TA:  J Clin Exp Dent     Country:  Spain    
Other Details:
Languages:  eng     Pagination:  e303-6     Citation Subset:  -    
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