[Pseudoxanthoma elasticum. Skin changes as a marker of systemic illness].

Angioid streaks were diagnosed in a 42-year-old woman. Since age 20 she 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 confirmed by a biopsy from lesional skin 22 years after the disease onset. Based on the present case and an analysis of the recent literature, the findings characteristic for pseudoxanthoma elasticum in the skin, eyes and cardiovascular system are delineated, the differential diagnosis 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.