Although calciphylaxis is a dermatologic diagnosis, most dermatology practitioners have never seen a case, yet most nephrology practitioners can identify calciphylaxis simply by viewing a lesion. Calciphylaxis is seen in 1%–4% of long-term dialysis patients, but can also be seen in patients with autoimmune diseases such as Crohn disease or lupus nephritis, cancer, hypercoagulable states and parathyroid disease.9
Traditional risk factors for calciphylaxis, besides uremia, are as follows: female sex, obesity, Caucasian race, high phosphorus and/or intact PTH level, maintaining on medications such as warfarin, vitamin D analogues, oral calcium and systemic corticosteroids.10
The natural course of the disease is indolent with a one-year survival rate of less than 50%, even with treatment.11 The pain that accompanies the lesion(s) cannot be overstressed. It is searing and continuous, as though a part of the body is clotted off. Treatment is multifactorial, and various specialists need to be involved to maximize the chance of wound healing. With the high rate of death and limb loss associated with calciphylaxis, the more aggressive the treatment, the better the chance of overall and limb survival.12
Prevention is vital since survival rates are poor. While calciphylaxis is more common among dialysis patients, cases that occur in patients without renal disease are more likely to be missed. A report on breast calciphylaxis that occurred after coronary artery bypass graft was recently published.13 At present, there are no specialized laboratory tests for diagnosing calciphylaxis; the patient’s phosphorus, calcium and/or intact PTH levels may be elevated, but just as often, they are in the normal- or low-range.14 Skin necrosis, typically referred to as metastatic calcification, can occur at normal levels.15 Even with normal readings, calcium and phosphate crystals can progressively accumulate in the small blood vessels of the fat tissue and skin. A high index of suspicion must be entertained when confronted with a suspicious lesion.
Mrs. V has been out of treatment for two years now. She spent two months in rehabilitation after her episode of calciphylaxis and was then weaned off of pain medications. She claims to be the only patient ever who was thrilled to have a bilateral mastectomy. She is now teaching phosphorus control to the other dialysis patients at the unit, and often states that she counts her blessings that she was one of the few who survived.
Kim Zuber, MS, PA-C, is a physician assistant practicing in Alexandria, Va.; Bill Bartow, PA-C, is the Clinical Director of the INOVA Wound Healing Center in Alexandria, Va.; and Jane Davis, DNP, practices in Birmingham, Ala.
1. Fisher AH, Morris, DJ. Pathogenesis of calciphylaxis: study of three cases with literature review. Hum Pathol. 1995;26:1055-1064.
2. Ross EA. Evolution of treatment strategies for calciphylaxis. Am J Nephrol. 2011;34:460-467. Available at content.karger.com/produktedb/produkte.asp?DOI=000332221&typ=pdf.
3. Singh RP, Derendorf H, Ross EA. Simulation-based sodium thiosulfate dosing strategies for the treatment of calciphylaxis. Clin J Am Soc Nephrol. 2011;6:1155-1159.
4. Raymond CB, Wazny LD. Sodium thiosulfate, bisphosphonates, and cinacalcet for treatment of calciphylaxis. Am J Health Syst Pharm. 2008;65:1419-1429.
5. Banerjee C, Woller SC, Holm JR, et al. Atypical calciphylaxis in a patient receiving warfarin then resolving with cessation of warfarin and application of hyperbaric oxygen therapy. Clin Appl Thromb Hemost. 2010;16:345-350.
6. Podymow T, Wherrett C, Burns KD. Hyperbaric oxygen in the treatment of calciphylaxis: a case series. Nephrol Dial Transplant. 2001;16:2176-2180. Available at ndt.oxfordjournals.org/content/16/11/2176.long.
7. National Kidney Foundation. NKF, K/DOQI clinical practice guidelines for chronic kidney disease: evaluation, classification, and stratification. Am J Kidney Dis. 2002;39:S1-S266.
8. Floege J. When man turns to stone: extraosseous calcification in uremic patients. Kidney Int. 2004;65:2447-2462. Available at www.nature.com/ ki/journal/v65/n6/full/4494554a.html.
9. Nigwekar SU, Wolf M, Sterns RH, Hix JK. Calciphylaxis from nonuremic causes: a systematic review. Clin J Am Soc Nephrol. 2008;3:1139-1143. Available at cjasn.asnjournals.org/content/3/4/1139.long.
10. Weenig RH, Sewell LD, Davis MD, et al. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol. 2007;56:569.
11. Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol. 2007;56:569-579.
12. Goel SK, Bellovich K, McCullough PA. Treatment of severe metastatic calcification and calciphylaxis in dialysis patients. Int J Nephrol. 2011;24:701603. Available at www.hindawi.com/journals/ijn/2011/701603/.
13. Cathenis K, Goossens D, Vertriest R, et al. Breast infarction due to calciphylaxis after coronary artery bypass grafting. Ann Thorac Surg. 2011;91:1603-1606.
14. Kalajian AH, Malhotra PS, Callen JP, Parker LP. Calciphylaxis with normal renal and parathyroid function: not as rare as previously believed. Arch Dermatol. 2009;145:451-458. Available at archderm.ama-assn.org/ cgi/content/full/145/4/451.
15. Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int. 2002;61:2210-2217. Available at www.nature.com/ki/journal/v61/n6/full/4493000a.html.
All electronic documents accessed July 15, 2012.
This article originally appeared on Clinical Advisor