Unusual case of proximal calciphylaxis without renal failure.

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.