Vascular Abnormalities Associated with Pseudoxanthoma Elasticum

Abstract

Evidence has been presented by several authors that the skin condition known as pseudoxanthoma elasticum may be associated with widespread abnormalities of the blood vessels of the limbs and viscera (Sandbacka

Arterial surgery in pseudoxanthoma elasticum

“…Arteriography in one study (Carlborg et al, 1959) demonstrated arterial occlusive changes frequently in the lower limbs, whereas others (Goodman et al, 1963) found such changes more prominent in the upper extremities. Calcification of peripheral arteries had been reported as early as the ninth year of life (Wolff, Stokes and Schlesinger, 1952). There was no hypertension in the present patient although it has been described in 22-5 % of cases (Eddy and Farber, 1962).…”

Clinical Pathologic Observations in Seudoxanthoma Elasticum

“…Since granules in the periphery of elastic fibers have been reported in other conditions and are frequently interpreted as an indication of active elastogenesis,'''' ^^ we believe that the increased amounts of elastic fibers and peripheral granules in these elastic fibers represent increased elastogenesis in pseudoxanthoma elasticum. (2) In all except Case 4, the elastic fibers were electron lucent. The presence of a dense matrix in the elastic fibers in one case (Fig.…”

Sideroelastosis pulmonum: Cause or Consequence of the Pulmonary Veno-Occlusive Disease?

“…Among possible causative factors of the elastic tissue degeneration, the authors dis cussed pathogenetic mechanisms similar to those in pseu doxanthoma elasticum. Reports of pulmonary vascular changes analogous to SP in pseudoxanthoma elastica have been published [24].…”