Classic Type Ehlers-Danlos Syndrome with Cutis Laxa-like Histopathologic Findings.
- Author:
Ui Kyung KIM
1
;
Hyun Min NAM
;
Kun PARK
;
Seok Don PARK
Author Information
1. Department of Dermatology, Wonkwang University School of Medicine, Iksan, Korea. sdpark@wonkwang.or.kr
- Publication Type:Case Report
- Keywords:
Cutis laxa;
Ehlers-Danlos syndrome
- MeSH:
Aged;
Cicatrix;
Connective Tissue;
Cutis Laxa;
Disease Susceptibility;
Ehlers-Danlos Syndrome;
Elastic Tissue;
Hemorrhage;
Hernia;
Humans;
Joints;
Nose;
Skin
- From:Korean Journal of Dermatology
2011;49(6):524-528
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Ehlers-Danlos syndrome is a connective tissue disorder that is characterized by hyper-extensible skin, hyper-mobile joints, fragile tissues, bleeding diathesis and poor healing of wounds with "cigarette paper" scar formation. Cutis laxa is also a disease of the elastic fibers of the connective tissue and characterized by loose skin folds over all parts of the body, a prematurely aged appearance, a hooked nose with a short colummella and umbilical/inguinal hernias. We report here on two patients who showed the symptoms of Ehlers-Danlos syndrome with histopathologically reduced and distorted elastic fibers, which is usually seen in cutis laxa.
