Calcific Uremic Arteriolopathy: An Underrecognized Entity

Abstract

Calcific uremic arteriolopathy (CUA), or calciphylaxis, is an uncommon and underrecognized disease that often occurs in the setting of chronic kidney disease or end-stage renal disease. It is characterized by small-vessel calcification, although many times it is associated with normal serum levels of calcium, phosphorus, and parathyroid hormone. The lesions appear as necrotic eschars, ulcerations, indurated nodules, and dry gangrene and are usually very painful. Diagnosis is based on clinical judgment and recognition of characteristic skin lesions. Biopsy can be performed but may be complicated by poor wound healing. Treatment of CUA involves rigorous wound care, strict control of mineral metabolism with avoidance of calcium and vitamin D analogs, and pain control. Other treatment options include sodium thiosulfate, hyperbaric oxygen therapy, daily hemodialysis using low-calcium dialysate, and bisphosphonates. Even with treatment, CUA is associated with significant morbidity and mortality. The patient in the case reported here had characteristic skin lesions and several risk factors for CUA, but diagnosis was delayed.