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The Multifactorial Pathogenesis of Calciphylaxis: A Case Report

Marta Turek, Joanna Stępniewska, Jacek Różański

(Department of Nephrology, Transplantology and Internal Medicine, Pomeranian Medical University, Szczecin, Poland)

Am J Case Rep 2021; 22:e930026

DOI: 10.12659/AJCR.930026

BACKGROUND: Calciphylaxis is a rare and lifethreatening syndrome characterized by small vascular calcifications, which lead to the occlusion of blood vessels and painful skin lesions with tissue necrosis. Although the disease can develop in a population without kidney failure, it is typically detected in patients receiving dialysis, with an increasing frequency ranging from 1% to 4%. Therefore, the disease is also known as calcific uremic arteriolopathy. The prognosis in patients with coexisting chronic kidney disease is very poor, with a 1-year mortality rate of up to 80%. Numerous risk factors for calciphylaxis have been described, such as obesity, diabetes mellitus, female sex, White race, overuse of calcium and vitamin D supplements, and vitamin K deficiency. The disease is often accompanied by disorders such as hyperphosphatemia, elevated parathyroid hormone level, and a deficiency of natural calcification inhibitors, such as fetuin-A and matrix Gla protein. However, not all patients with calciphylaxis have the abnormalities described above, suggesting that the pathogenesis of calciphylaxis is multifactorial and unfortunately still uncertain.
CASE REPORT: We report a case of calciphylaxis in a 52-year-old White woman with multiple comorbidities and on chronic hemodialysis treatment, who presented with severe subcutaneous painful nodules and necrotic ulcers on both legs.
CONCLUSIONS: Although the prognosis of this rare and underrecognized disease is poor, an early diagnosis and interdisciplinary treatment including pain relief, wound care, appropriate nutritional support, correction of mineral parameters, administration of sodium thiosulphate, and adequate hemodialysis therapy can improve patient quality of life.

Keywords: calciphylaxis, Renal Dialysis, renal insufficiency

This paper has been published under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.
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