ABSTRACT

Ehlers-Danlos syndrome is a heritab/e connective tissue disorder characterized clinically by joint laxity, ski n hyperextensibility and fragility. It is classified in to II different subgroups according to clinical, genetic, biochemical propertıes. However, same oj the patıents may not fit completely to one of the se subgroups. 42 year old man presented to our polyclinic because of having Iate healing wounds and large scars of the healed wounds. Atrophıc scars on the face, back, arms, legs, and dorsum of the feet, hyperflexibilıty oj joints, and cutis hyperelastica was jound of hıs dermatological examination. EDS diaonosis was established after the elinical and laboratory investigation. However, we could not demonstrate the inheritance type because his relatives dıdn't have similar signs and symptoms. Our patient's disease was suitable clinically to EDS type I and VIII subgroup properties in adition to autosomal damınant inheritance. Heartfailure and cardiomyopathy was detected during the investıgationfor the breathlessness complaint ajter two years oj the dıagnosis.