Abstract
Pseudoxanthoma elasticum (PXE) is a multi-systemic, genetic disorder of connective tissue characterized by progressive calcification and fragmentation of elastic fibers in skin, retina and cardiovascular system. A young patient reported to urologist in emergency with azotemia secondary to acute bilateral obstructive uropathy. Upon successful management, he was found to have full-blown skin and eye manifestations and was evaluated thoroughly regarding extent and potential systemic complications of Pseudoxanthoma elasticum.
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