Abstract
Pseudoxanthoma elasticum (PXE) is a rare metabolic disease characterized by reduced plasma pyrophosphate (PPi) concentration, causing progressive soft tissue calcification represented by skin lesions, central vision lost and peripheral artery disease. PXE is currently incurable. Previous reports have shown early high failure after revascularization by unknown mechanism. Reports of oral PPi administration have shown to decrease tissue calcification in a murine model of PXE. We report the outcome of one patient treated with oral PPi and further operated for critical limb ischemia. During the one-year follow-up the operated area has not re-occluded and there have been no significant side effects.
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