Korean J Med.  2015 Oct;89(4):474-477. 10.3904/kjm.2015.89.4.474.

Pseudoxanthoma Elasticum-Like Disorder with Multiple Coagulation Factor Deficiency in a Patient with Crohn's Disease

Affiliations
  • 1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. khlee2@amc.seoul.kr

Abstract

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.

Keyword

Pseudoxanthoma elasticum-like disorder with multiple coagulation factor deficiency; Crohn disease

MeSH Terms

Adult
Arteries
Blood Coagulation Factors*
Connective Tissue
Crohn Disease*
Female
Gastrointestinal Tract
Humans
Intestine, Small
Malnutrition
Pseudoxanthoma Elasticum
Retina
Skin
Blood Coagulation Factors
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