Korean J Hematol 1995; 30(2):
Published online June 30, 1995
© The Korean Society of Hematology
재생불량성 빈혈과 동반된 선천성 이각화증 1례
전진종, 김병훈, 강호정, 이경희, 현명수, 최종수
영남대학교 의과대학 내과학교실,
영남대학교 의과대학 피부과학교실
A Case of Dyskeratosis Congenita with Aplastic Anemia
Dyskeratosis conlgenita is a rare congenital disorder characterized by the triad of
reticular pigmentation of the skin, dystrophic nails, leukoplakia of the mucous membrane
and often associated with aplastic anemia.
We have experienced a 15-year-old boy who had reticular pigmentation of the skin,
dystrophic changes of the fingers and toe nails. The tongue was smooth and lingual
papillae disappeared with formation of adherent white leukoplakic patches.
Laboratory data revealed pancytopenia, significantly decreased bone marrow cellular
elements with increased fat cells.
Keywords Aplastic anemia, Hyperpigmentation of skin, Leukoplakia, Nail dystrophy
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Korean J Hematol 1995; 30(2): 321-327
Published online June 30, 1995
Copyright © The Korean Society of Hematology.
재생불량성 빈혈과 동반된 선천성 이각화증 1례
전진종, 김병훈, 강호정, 이경희, 현명수, 최종수
영남대학교 의과대학 내과학교실,
영남대학교 의과대학 피부과학교실
A Case of Dyskeratosis Congenita with Aplastic Anemia
Jin Jong Jeon, Byung Hoon Kim, Ho Jung Kang, Kyung Hee Lee, Myung Soo Hyun, Jong Soo Choi
Department of Internal Medicine, Dermatology, Yeungnam University College of Medicine, Taegu, Korea
Abstract
Dyskeratosis conlgenita is a rare congenital disorder characterized by the triad of
reticular pigmentation of the skin, dystrophic nails, leukoplakia of the mucous membrane
and often associated with aplastic anemia.
We have experienced a 15-year-old boy who had reticular pigmentation of the skin,
dystrophic changes of the fingers and toe nails. The tongue was smooth and lingual
papillae disappeared with formation of adherent white leukoplakic patches.
Laboratory data revealed pancytopenia, significantly decreased bone marrow cellular
elements with increased fat cells.
Keywords: Aplastic anemia, Hyperpigmentation of skin, Leukoplakia, Nail dystrophy
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