Calciphylaxis with a protracted course in a patient with end-stage renal failure

Abstract

Calciphylaxis is a rare condition with a high mortality involving mural calcification of small vessels and occurring primarily in patients with end-stage renal disease. Most cases are characterized by rapid progression of tender subcutaneous nodules or cutaneous plaques to epidermal necrosis, with death in the majority of cases occurring from sepsis. The condition is still poorly understood and the pathogenesis not known. We report a case of calciphylaxis following an unusually benign course. Our patient was a 63-year-old Chinese woman with end-stage renal failure undergoing peritoneal dialysis who presented with itchy pruriginous rashes on her limbs and trunk with subsequent development of eschars and ulceration. Biopsy confirmed the presence of calcification within vessel walls in the subcutis. She was managed conservatively and refused a parathyroidectomy. Surprisingly, her disease did not worsen but remained stable. She died from an acute myocardial infarction 5 months after presenting to our clinic.