title: Zinsser-Cole-Engmann syndrome: A rare case report with literature review.
abstract: 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.affiliation: Professor and Head of Department. Department of Oral Medicine and Radiology, Government Dental College and Hospital, Srinagar.
authors.forename: Altaf H
authors.initials: AH
authors.lastname: Chalkoo
authors.affiliation: Post Graduate Student. Department of Oral Medicine and Radiology, Government Dental College and Hospital, Srinagar.
authors.forename: Vibhuti
authors.initials: V
authors.lastname: Kaul
authors.affiliation: Assistant Professor. Department of General Pathology, Government Medical College, Srinagar.
authors.forename: Lateef A
authors.initials: LA
authors.lastname: Wani