학술논문
크론병과 동반된 탄력섬유성위황색종양 질환
Pseudoxanthoma Elasticum-Like Disorder with Multiple Coagulation Factor Deficiency in a Patient with Crohn`s Disease
Pseudoxanthoma Elasticum-Like Disorder with Multiple Coagulation Factor Deficiency in a Patient with Crohn`s Disease
Document Type
Article
Author
강병주 / Byeong Zu Ghang; 이지완 / Ji Wan Lee; 김좌훈 / Jwa Hoon Kim; 서유리 / Yu Ri Seo; 김민철 / Min Chul Kim; 정영진 / Yeong Jin Jeong; 이규형 / Kyoo Hyung Lee
Source
대한내과학회지 (Korean J Med) / The Korean Journal of Medicine (Korean J Med). Oct 31, 2015 89(4):474
Subject
Language
Korean
ISSN
1738-9364
Abstract
크론병에 의한 만성 염증으로 흡수 장애는 잘 알려져 있지만 비타민K의 이용에 장애가 있는 희귀한 유전병인 탄력섬 유성위황색종양 질환이 동반되어 응고장애의 악화를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
Pseudoxanthoma elasticum (PXE)-like disorder with multiple coagulation factor deficiency is a genetic disease characterized by dystrophic mineralization of the connective tissue in multiple organs such as the skin, arteries, and retina. Patients with this disorder have excessive folding and sagging of the skin with loss of recoil, as well as coagulopathy. Crohn’s disease is a chronic inflammatory condition of the gastrointestinal tract, which primary affects the small intestine, often leading to malnutrition. Here, we describe the case of a 25-year-old woman who presented with these diseases. We found that nutrient deficiency due to long-term Crohn’s disease aggravated the coagulopathy associated with PXE-like disorder. (Korean J Med 2015;89:474-477)
Pseudoxanthoma elasticum (PXE)-like disorder with multiple coagulation factor deficiency is a genetic disease characterized by dystrophic mineralization of the connective tissue in multiple organs such as the skin, arteries, and retina. Patients with this disorder have excessive folding and sagging of the skin with loss of recoil, as well as coagulopathy. Crohn’s disease is a chronic inflammatory condition of the gastrointestinal tract, which primary affects the small intestine, often leading to malnutrition. Here, we describe the case of a 25-year-old woman who presented with these diseases. We found that nutrient deficiency due to long-term Crohn’s disease aggravated the coagulopathy associated with PXE-like disorder. (Korean J Med 2015;89:474-477)