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KMID : 0882420150890040474
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Korean Journal of Medicine
2015 Volume.89 No. 4 p.474 ~ p.477
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Pseudoxanthoma Elasticum-Like Disorder with Multiple Coagulation Factor Deficiency in a Patient with Crohn’s Disease
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Kang Byung-Ju
Lee Ji-Wan
Kim Jwa-Hoon
Seo Yu-Ri
Kim Min-Chul
Jeong Yeong-Jin
Lee Kyoo-Hyung
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Abstract
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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.
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KEYWORD
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Pseudoxanthoma elasticum-like disorder with multiple coagulation factor deficiency, Crohn disease
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