Unusual Case of Proximal Calciphylaxis without Renal Failure

Abstract

Background:

Calciphylaxis is a rare syndrome of vascular calcification with subsequent cutaneous and tissue necrosis. It usually manifests as a complication of end-stage renal failure, affecting 1 to 4% of long-term dialysis patients. Very exceptionally, it can occur without chronic renal failure.

Objective:

The goal of this study was to discuss an exceptional case of extensive calciphylaxis in the absence of chronic renal failure and its successful management.

Methods:

We present a case of a 31-year-old woman with extensive proximal, ulcerated calciphylaxis without associated chronic renal failure. Our patient had quite a few risk factors associated with the pathogenesis of calciphylaxis, such as obesity, malnutrition, and a transient episode of acute renal failure.

Results:

She was successfully treated with sodium thiosulfate, extensive wound débridement (more than 30% total body surface), and subsequent skin grafts. The patient has miraculously survived this often fatal condition.

Conclusion:

Calciphylaxis can occur even in the absence of chronic renal failure. This often fatal condition can be managed successfully with a combination of aggressive wound control and the fairly newly described sodium thiosulfate therapy.