Ehlers-danlos syndrome associated with cardiomyopathy hypertrophic obstructive

Ehlers-danlos syndrome is a rare clinical condition caused by a genetic change that results in the formation of structurally or functionally altered collagen. the clinical manifestations are varied,being the most obvious skin hypermotility and increased joint fl exibility,although other systems - such as cardiovascular,respiratory and neurological - may also be affected. this paper presents the report of a patient who sought medical attention with complaints of atypical chest pain. clinical evaluation enabled hypothetical diagnosis of hypertrophic obstructive cardiomyopathy and ehlers-danlos syndrome. initial electrocardiogram,echocardiogram and 24 hours holter allowed the confirmation of the first hypothesis. a skin biopsy performed later associated clinical data and confirmed the second hypothesis. © 2015 by anais brasileiros de dermatologia.