Dyskeratosis congenita

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Dyskeratosis congenita
ICD-10 Q82.8
ICD-9 757.39
OMIM 305000
DiseasesDB 30105
eMedicine derm/111 
MeSH D019871

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]


Overview

Dyskeratosis congenita (DKC), also called Zinsser-Cole-Engman syndrome,[1][2]:570 is a rare progressive congenital disorder that in some ways resembles premature aging (similar to progeria). The disease mainly affects the integumentary system, the organ system that protects the body from damage, with a major consequence being anomalies of the bone marrow.

  1. Online Mendelian Inheritance in Man (OMIM) 305000
  2. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.