Pseudoxanthomaelasticum with congestive heart failure: A case report

Pseudoxanthomaelasticum (PXE) is a rare systemic disease of connective tissue primarily affecting the skin, retinae, and cardiovascular system. Clinically it has high hetrogenicity in age of onset, extent and severity of disease. Its cardiovascular effect has a wide clinical spectrum extending from mental fatigue to early death from myocardial infarction. Very rarely it may present with gastrointestinal haemorrhage. It has no specific treatment. However, adjustments of the life stile are important to reduce morbidity. Moreover, first degree relatives should be carefully examined for cutaneous and ophthalmic features of this disease. Here we describe one case of Pseudoxanthoma Elasticum. Sudan Journal of Medical Sciences Vol. 1(1) 2006: 62-6