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Images of Memorable Cases: Case 49

Module by: Herbert L. Fred, MD, Hendrik A. van Dijk

Case_49-pres1-1.jpg Case_49-pres1-2.jpg

This 42-year-old woman with known angina pectoris experienced black stools and hematemesis of one day’s duration. On physical examination, she had greatly diminished femoral, popliteal, and pedal pulses together with angioid streaks in both optic fundi.

49. Pseudoxanthoma elasticum

A disorder of connective tissue characterized by degeneration and calcification of elastic fibers in the skin, eyes, and cardiovascular system. The cutaneous changes resemble “plucked chicken skin” and typically appear in the neck and flexural areas. Angioid streaks represent breaks in the elastic lamina of Bruch’s membrane beneath the retina. Accelerated atherosclerosis at an early age is common. Gastrointestinal bleeding sometimes heralds the disease and presumably results from altered elastic tissue in the walls of small mucosal arteries.

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