Korean J Dermatol.
2011 Jun;49(6):524-528.
Classic Type Ehlers-Danlos Syndrome with Cutis Laxa-like Histopathologic Findings
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- 1Department of Dermatology, Wonkwang University School of Medicine, Iksan, Korea. sdpark@wonkwang.or.kr
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.
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