Although histopathologic studies have shown that angioid streaks of the retina and pseudoxanthoma elasticum are only part of a generalized elastic tissue degeneration, which includes the arterial tree, the clinical relationship to arterial disease is less well known. This paper adds 10 patients to those previously reported in the literature, all of whom showed clinical evidence of arterial disease, especially marked in the lower extremities. Symptoms suggestive of vascular disease may be absent or extreme, depending upon the location of the vessel and the severity of the involvement. When the arteries of the extremities are affected, the patient may notice paresthesias, numbness, chilling, or intermittent claudication. Objective signs may be obvious or detectable only by careful examination. Pulses may be weak or absent. Pulse waves recorded on an oscillometer are ofter reduced or even absent. Vasodilatation tests may demonstrate reduced blood flow. Roentgen examination of the extremities often shows calcification