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PubMed · 8115902

Systemic calciphylaxis.

Abstract

Systemic calciphylaxis is a rare, poorly understood syndrome of progressive ischemic necrosis, usually associated with hyperparathyroidism. The combination of hyperparathyroidism, usually secondary or tertiary, and chronic renal failure seems to produce a particular biochemical environment conducive to the development of an unusual progressive form of ischemia. Early recognition and diagnosis, appropriate therapy including wound care, and expeditious parathyroidectomy often will halt the progression of the disease, minimize limb loss, and prevent subsequent sepsis and death.

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BibTeXRIS

R B Kent, R T Lyerly. 1994. Systemic calciphylaxis.. https://doi.org/10.1097/00007611-199402000-00029

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Calciphylaxis with histologic changes of pseudoxanthoma elasticum.

Calciphylaxis is a rare condition of widespread calcification of tissues and blood vessels with accompanying vascular thrombosis and ischemic necrosis. Most cases develop in association with hyperparathyroidism in patients with chronic renal failure. Pseudoxanthoma elasticum (PXE) is a hereditary condition of abnormal elastic tissue structure that leads to widespread abnormalities of the skin, retina, and visceral organs. Histologic changes of PXE have been observed as coincidental findings in several conditions such as following trauma to the skin manifest as isolated plaques often in scars. We observed histologic findings of PXE in a patient with chronic renal failure who developed fatal calciphylaxis. Complete evaluation failed to reveal evidence of systemic findings of PXE. Histologic changes of PXE may be seen in patients with calciphylaxis as a coincidental finding. Rapidly developing soft tissue calcification may lead to the expression of the characteristic histopathologic findings of PXE without evidence of classic clinical manifestations of PXE. Calciphylaxis should be added to the list of disorders that may lead to microscopic PXE-like changes.

Calciphylaxis