Abstract
Calciphylaxis is a rare and highly morbid disorder characterized by painful skin lesions and vascular calcification (Hayashi, Clin Exp Nephrol 17(4):498–503, 2013). Despite being described in case reports for over 50 years, the disease and its pathogenesis remain to this day poorly understood (Rees and Coles, Br Med J 2(5658):670–672, 1969). Originally recognized as a condition found among patients with chronic renal failure on dialysis, patients with normal kidney function can also be afflicted, although much less commonly (Nigwekar et al., Am J Kidney Diseases 2015). On presentation the skin lesions typically appear as tender nodules that progress to ulceration and necrosis. The lack of defining diagnostic features complicates efforts to distinguish calciphylaxis from other conditions that often mimic it. Biopsy of lesions typically reveals calcium–phosphate deposits in small and medium sized cutaneous arteries (Biller et al., Acta Clin Belg 67(6):442–444, 2012). Therapy is largely medical and limited to pain control, wound care, and management of septic complications that are the leading causes of high mortality rates. Parathyroidectomy is indicated for those patients with hyperparathyroidism, and in select patients, wound debridement may be necessary.
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Lee, D.T., Owen, R.P. (2017). Calciphylaxis. In: Stack, Jr., B., Bodenner, D. (eds) Medical and Surgical Treatment of Parathyroid Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-26794-4_32
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DOI: https://doi.org/10.1007/978-3-319-26794-4_32
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