Classic Type Ehlers-Danlos Syndrome with Cutis Laxa-like Histopathologic Findings / 대한피부과학회지

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.