Abstract

Calciphylaxis is a condition of calcium and phosphate metabolism dysregulation which leads to calcification and thrombosis of the subcutaneous vasculature with subsequent skin necrosis. Calciphylaxis is most often a disease of the subcutaneous tissue and is most common in areas of dense adipose tissue. However, there have been numerous reports of extracutaneous manifestations in patients with diagnosed calciphylaxis. Herein we report clinicopathologic data of 7 autopsy patients with an antemortem diagnosis of calciphylaxis. Clinical and demographic features were reported including associated risk factors and antemortem treatments. Autopsy slides were re-reviewed for findings of calciphylaxis (extravascular and vascular calcification of the small-medium sized vessels with or without necrosis and thromboses) in other organ systems. All 7 patients developed antemortem skin lesions, occurring on average 37 days prior to death with a range of 6-120 days. Calciphylaxis was present on the death certificate as a contributing cause in 4 cases. All but one decedent was female (6 cases) which was the most common risk factor along with moderate-severe chronic kidney disease (6 cases) followed by chronic warfarin use (5 cases). Histologic examination of the autopsy tissue identified cutaneous findings of calciphylaxis in all 7 cases. Extracutaneous calcifications were noted in 5 cases with the kidneys most frequently involved (5 cases) with vascular and tubular calcifications noted. The pancreas was involved in 3 cases with large and small vessels involvement and calcifications of the fat in one case. Other sites noted include lung, mesentery, adrenal gland, and a paratracheal lymph node. These findings highlight the fact that calciphylaxis is a systemic disease with the ability to involve extracutaneous organs.

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