Early stages of calciphylaxis: are skin biopsies the answer?

Calciphylaxis, nowadays called calcific uremic arteriolopathy (CUA), is a rare but life-threatening syndrome characterized by systemic medial calcification and arterial occlusion of the arterioles, leading to skin necrosis. Actually, the pathogenesis of CUA is complex and poorly understood. The vast majority of published cases presented with ulcers. We present a 71-year-old male who was referred to the Nephrology Department for evaluation and therapy for refractory edema of both legs. There were no subcutaneous plaques, ulcers or other focal lesions. We performed a deep skin biopsy of the thigh. After the biopsy, the patient developed necrosis around the sampling location. Diagnosing CUA in the early stages of the disease, however, is challenging. Should a skin biopsy be performed in the early stages of CUA in patients without ulcerations, knowing the risk of inducing ulcerations, or should an intensive treatment - after considering clinical manifestations, other noninvasive diagnostic tools (bone scan, X-ray mammography technique) and laboratory tests - be started? Although there are no specific diagnostic laboratory tests for CUA and the clinical manifestations of CUA are similar to those of other disorders, a skin biopsy is not routinely recommended to confirm the diagnosis of early-stage calciphylaxis.