Calciphylaxis in a Peritoneal Dialysis Patient

Calciphylaxis, also referred to as Calcific Uremic Arteriolopathy, is a rare and life-threatening disorder that should be considered as a differential diagnosis in end-stage renal disease (ESRD) patients presenting with painful skin lesions. It presents with livedo reticularis and painful subcutaneous nodules which ultimately progress to skin ischemia and necrosis. Although skin lesions tend to dominate the clinical presentation, this disorder is likely a systemic disorder with widespread calcifications involving the vasculature of the skeletal muscles, intestines, mesentery, optic nerves and lungs. Although it is largely described in patients with ESRD, it may also be seen with less advanced chronic kidney disease, acute kidney injury, and even those with normal kidney function.

In addition to CKD, other risk factors include female sex, white race, obesity, diabetes mellitus, hypercalcemia, hyperphosphatemia, metastatic cancers, recurrent hypotension, and warfarin therapy. We present the case of a 47-year-old white male on peritoneal dialysis who presented to the hospital with dusky discoloration of his skin with multiple well-demarcated black, necrotic painful skin lesions on his fingers and toes for three weeks. Given the laboratory and imaging findings there was a high clinical suspicion for calciphylaxis.

Keywords: Calciphylaxis; Calcific uremic arteriolopathy; End-stage renal disease (ESRD); Peritoneal Dialysis; Sodium thiosulfate; Vascular calcifications; Livedo reticularis; Matrix gla protein (MGP)