PSEUDOXANTHOMA ELASTICUM - SKIN-LESIONS A S A SIGNAL TO A SYSTEMIC-DISEASE

Angioid streaks were diagnosed in a 42-year-old woman. Since age 20 sh e had developed circumscribed atrophic yellow-grey lesions on her neck , axillae and other flexural sites. In spite of highly characteristic skin changes, the diagnosis of pseudoxanthoma elasticum was first conf irmed by a biopsy from lesional skin 22 years after the disease onset. Based on the present case and an analysis of the recent literature, t he findings characteristic for pseudoxanthoma elasticum in the skin, e yes and cardiovascular system are delineated, the differential diagnos is of the skin lesions, as well as the pathogenesis and the prognosis are discussed, and the new classification of pseudoxanthoma elasticum, based on major and minor criteria, is described.