Abstract

Calciphylaxis is a severe, often lethal condition frequently seen in patients with renal disease who present with varying degrees of skin necrosis.  Although poorly understood and rarely reported, more indolent cases of histologic calciphylaxis with only clinically delayed skin necrosis or lack of eschar formation have been termed “protracted calciphylaxis.”  Herein, we present a case of histologically apparent calciphylaxis with a protracted clinical course in a patient with a history of peripheral artery disease and poorly controlled diabetes but without calcium derangements or apparent kidney disease. Over the course of a year, the patient reported gradually worsening tenderness over the left lower extremity and was eventually admitted to the hospital with severe leg pain. Examination showed a large, hyperpigmented, tender, indurated plaque with associated vessel calcification on computed tomography angiography. Histologic examination showed dermal to subcutaneous fibrosis with septal thickening, lobular fat necrosis, and granuloma formation. Von Kossa positive calcifications were noted in large subcutaneous vessels, eccrine coils, and smaller vessels within fatty lobules. Infectious studies were negative. To date, the patient continues to report lower extremity pain but remains free of the more dire sequela associated with calciphylaxis. Calciphylaxis continues to be a diagnostic challenge and – as this case demonstrates – may show a spectrum of clinical presentations. This case report offers additional evidence of the protracted form and highlights the need for more research into the pathogenesis and natural history of calciphylaxis.