BACKGROUND AND OBJECTIVES: Calciphylaxis, or calcific uremic arteriolopathy, is 
a well-described entity in end-stage kidney disease and renal transplant 
patients; however, little systematic information is available on calciphylaxis 
from nonuremic causes. This systematic review was designed to characterize 
etiologies, clinical features, laboratory abnormalities, and prognosis of 
nonuremic calciphylaxis.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: A systematic review of literature 
for case reports and case series of nonuremic calciphylaxis was performed. Cases 
included met the operational definition of nonuremic 
calciphylaxis-histopathologic diagnosis of calciphylaxis in the absence of 
end-stage kidney disease, renal transplantation, or acute kidney injury 
requiring renal replacement therapy.
RESULTS: We found 36 cases (75% women, 63% Caucasian, aged 15 to 82 yr) of 
nonuremic calciphylaxis. Primary hyperparathyroidism, malignancy, alcoholic 
liver disease, and connective tissue disease were the most common reported 
causes. Preceding corticosteroid use was reported for 61% patients. Protein C 
and S deficiencies were seen in 11% of patients. Skin lesions were 
morphologically similar to calcific uremic arteriolopathy. Mortality rate was 
52%, with sepsis being the leading cause of death.
CONCLUSION: Calciphylaxis should be considered while evaluating skin lesions in 
patients with predisposing conditions even in the absence of end-stage kidney 
disease and renal transplantation. Nonuremic calciphylaxis is reported most 
often in white women. Mineral abnormalities that are invoked as potential causes 
in calcific uremic arteriolopathy are often absent, suggesting that 
heterogeneous mechanisms may contribute to its pathogenesis. Nonuremic 
calciphylaxis is associated with high mortality, and there is no known effective 
treatment.