Calciphylaxis (Calcific Uremic Arteriolopathy)


Calciphylaxis is a syndrome of subcutaneous vascular calcification resulting in painful ulcers on the legs, thighs, or abdomen. The disease develops almost exclusively in patients with end-stage renal disease, and is frequently fatal due to infectious complications. Calciphylaxis was described in 1962 by Hans Selye. He made rats hypercalcemic with “sensitizers” vitamin D or parathyroid hormone. “Challengers” were then administered. These included skin injury by hair-plucking, or injections of various sorts. Injured areas calcified and became ulcerated. Injection of “challengers” intravenously resulted in systemic necrotic lesions associated with tissue calcification (1). The concept of calciphylaxis has been imperfectly applied to cutaneous ulcerations that develop in patients with end-stage renal disease due to vascular calcification and subsequent occlusion. While this clinical scenario has some parallels with Selye’s experiments, his experimental subjects were not uremic, and the calcifications were not vascular. Therefore, “calciphylaxis” does not accurately describe the syndrome. Because this disease does not conform to the model of calciphylaxis as described by Selye, many advocate eliminating that label in favor of “calcific uremic arteriolopathy.”


Secondary Hyperparathyroidism Parathyroid Hormone Level Primary Hyperoxaluria Indurated Plaque Secondary Hyperparathy 
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  1. 1.
    Selye H. Calciphylaxis. Chicago, IL: University of Chicago Press, 1962.Google Scholar
  2. 2.
    Barri YM, Graves GS, Knochel JP. Calciphylaxis in a patient with Crohn’s disease in the absence of end-stage renal disease. Am J Kidney Dis 1997;29:773–776.PubMedGoogle Scholar
  3. 3.
    Fader DJ, Kang S. Calciphylaxis without renal failure. Arch Dermatol 1996;132:837–838.PubMedCrossRefGoogle Scholar
  4. 4.
    Chavel SM, Taraszka KS, Schaffer JV, Lazova R, Schechner JS. Calciphylaxis associated with acute, reversible renal failure in the setting of alcoholic cirrhosis. J Am Acad Dermatol 2004;50:S125–S128.PubMedCrossRefGoogle Scholar
  5. 5.
    Mastruserio DN, Nguyen EQ, Nielsen T, Hessel A, Pellegrini AE. Calciphylaxis associated with metastatic breast carcinoma. J Am Acad Dermatol 1999;41:295–298.PubMedCrossRefGoogle Scholar
  6. 6.
    Mirza I, Chaubay D, Gunderia H, Shih W, El-Fanek H. An unusual presentation of calciphylaxis due to primary hyperparathyroidism. Arch Pathol Lab Med 2001;125:1351–1353.PubMedGoogle Scholar
  7. 7.
    Llach F. Calcific uremic arteriolopathy (calciphylaxis): An evolving entity? Am J Kidney Dis 1998;32:514–518.PubMedGoogle Scholar
  8. 8.
    Mawad HW, Sawaya BP, Sarin R, Malluche HH. Calcific uremic arteriolopathy in association with low turnover bone disease. Clin Nephrol 1999;52:160–166.PubMedGoogle Scholar
  9. 9.
    Vattikuti R, Towler DA. Osteogenic regulation of vascular calcification: An early perspective. Am J Physiol Endocrinol Metab 2004;286:E686–E696.PubMedCrossRefGoogle Scholar
  10. 10.
    Giachelli CM. Vascular calcification mechanisms. J Am Soc Nephrol 2004;15:2959–2964.PubMedCrossRefGoogle Scholar
  11. 11.
    Wilmer WA, Magro CM. Calciphylaxis: emerging concepts in prevention, diagnosis, and treatment. Semin Dialysis 2002;15:172–186.CrossRefGoogle Scholar
  12. 12.
    Jono S, Nishizawa Y, Shioi A, Morii H. 1,25-Dihydroxyvitamin D3 increases in vitro vascular calcification by modulating secretion of endogenous parathyroid hormone-related peptide. Circulation 1998;98:1302–1306.PubMedGoogle Scholar
  13. 13.
    Munavalli F, Reisenauer A, Moses M, Kilroy S, Arbiser JL. Weight loss-induced calciphylaxis: potential role of matrix metalloproteinases. J Dermatol 2003;30:915–919.PubMedGoogle Scholar
  14. 14.
    Janigan DT, Hirsch DJ, Klassen GA, MacDonald AS. Calcified subcutaneous arterioles with infarcts of the subcutis and skin (“calciphylaxis”) in chronic renal failure. Am J Kidney Dis 2000;35:588–597PubMedGoogle Scholar
  15. 15.
    Wilmer WA, Voroshilova O, Singh I, Middendorf DF, Cosio FG. Transcutaneous oxygen tension in patients with calciphylaxis. Am J Kidney Dis 2001;37:797–806.PubMedCrossRefGoogle Scholar
  16. 16.
    Mehta RL, Scott G, Sloand JA, Francis CW. Skin necrosis associated with acquired protein C deficiency in patients with renal failure and calciphylaxis. Am J Med 1990;88:252–257.PubMedCrossRefGoogle Scholar
  17. 17.
    Karpman E, Das S, Kurzrock EA. Penile calciphylaxis: analysis of risk factors and mortality. J Urol 2003;169:2206–2209.PubMedCrossRefGoogle Scholar
  18. 18.
    Hafner J, Keusch G, Wahl C, et al. Uremic small-artery disease with medial calcification and intimal hyperplasia (so-called calciphylaxis): A complication of chronic renal failure and benefit from parathyroidectomy. J Am Acad Dermatol 1995;33:954–962.PubMedCrossRefGoogle Scholar
  19. 19.
    Essary LR, Wick MR. Cutaneous calciphylaxis: An underrecognized clinicopathologic entity. Am J Clin Pathol 2000;113:280–287.PubMedCrossRefGoogle Scholar
  20. 20.
    Edsall LC, English JC, Patterson JW. Calciphylaxis and metastatic calcification associated with nephrogenic fibrosing dermopathy. J Cutan Pathol 2004;31:247–253.PubMedCrossRefGoogle Scholar
  21. 21.
    Don BR, Chin AI. A strategy for the treatment of calcemic uremic arteriolopathy (calciphylaxis) employing a combination of therapies. Clin Nephrol 2003;59:463–470.PubMedGoogle Scholar
  22. 22.
    Basile C, Montanaro A, Masi M, Pati G, De Maio P, Gismondi A. Hyperbaric oxygen therapy for calcific uremic arteriolopathy: A case series. J Nephrol 2002;15:676–680.PubMedGoogle Scholar
  23. 23.
    Price PA, Omid N, Than TN, Williamson MK. The amino bisphosphonate ibandronate prevents calciphylaxis in the rat at doses that inhibit bone resorption. Calcif Tissue Int 2002;71:356–363.PubMedCrossRefGoogle Scholar
  24. 24.
    Monney P, Nguyen QV, Perroud H, Descombes E. Rapid improvement of calciphylaxis after intravenous pamidronate therapy in a patient with chronic renal failure. Nephrol Dial Transplant 2004;19:2130–2132.PubMedCrossRefGoogle Scholar
  25. 25.
    Cicone JS, Petronis JB, Embert CD, Spector DA. Successful treatment of calciphylaxis with intravenous sodium thiosulfate. Am J Kidney Dis 2004;43:1104–1108.PubMedCrossRefGoogle Scholar
  26. 26.
    Sewell LD, Weenig RH, Davis MDP, McEvoy MT, Pittelkow MR. Low-dose tissue plasminogen activator for calciphylaxis. Arch Dermatol 2004;140:1045–1048.PubMedCrossRefGoogle Scholar

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