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Calciphylaxis -- Recent Reports on Managing This Life-Threatening Dermatosis

Many widely read journals have recently covered calciphylaxis. W.G. Goodman, among others, prefers the designation calcific uremic arteriolopathy because it better describes the pathologic feature of occlusion of vessels presumed to be secondary to calcium deposition. This disease occurs primarily in hemodialysis patients, often those with newly diagnosed renal failure. Classic teaching suggests a relation to secondary hyperparathyroidism, and the dermatology literature posits a survival advantage for patients treated with parathyroidectomy. Girotto et al. reviewed the cases of 13 calciphylaxis patients, 6 of whom underwent parathyroidectomy and experienced better wound healing and longer survival than did 7 patients treated with medical therapy; nevertheless, a high proportion of patients in both groups died.

In case-control studies, Mazhar et al. found that obese women with elevated phosphorus levels, elevated alkaline phosphatase levels, and low albumin levels were at the greatest risk for calciphylaxis, but did not find calcium levels, parathyroid hormone levels, and calcium-phosphorus products to be risk factors. These investigators also found an 8-fold greater risk for death when calciphylaxis was present. Ahmed et al. report similar findings, except they did identify a correlation between calcium-phosphorus products and risk for calciphylaxis. They also found that osteopontin, a protein normally made by osteoblasts, is expressed by smooth muscle cells in the vascular endothelium and may account for deposition of calcium within the vessel walls.

F. Llach, an editorialist, recommends parathyroidectomy only for patients with elevated parathyroid hormone levels and recommends use of noncalcium-containing phosphate binders and low-calcium dialysis. Lastly, although this disease is associated with renal failure, a case report by Riegert-Johnson et al. reminds us that not all patients with this condition have renal failure.

Comment: Calciphylaxis is not uncommon. Within the last 3 months, roughly half a dozen such patients have been seen in my practice. All patients were on dialysis and had elevated parathyroid hormone levels. Not all were women, and not all have undergone parathyroidectomy. At times we have had difficulty establishing the diagnosis histopathologically; I am beginning to believe that diagnosis may be established by demonstrating vascular calcification in the soft tissues under radiographic examination. Management is controversial, but some evidence supports the early use of parathyroidectomy when secondary hyperparathyroidism is evident. It is to be hoped that early diagnosis and treatment will improve survival.

— Jeffrey P. Callen, MD

Published in Journal Watch Dermatology January 15, 2002

Citation(s):

Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 31-2001: A 70-year-old woman with end-stage renal disease and cutaneous ulcers. N Engl J Med 2001 Oct 11; 345:1119-24.

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Goodman WG. Vascular calcification in chronic renal failure. Lancet 2001 Oct 6; 358:1115-6.

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Girotto JA et al. Parathyroidectomy promotes wound healing and prolongs survival in patients with calciphylaxis from secondary hyperparathyroidism. Surgery 2001 Oct; 130:645-50.

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Mazhar AR et al. Risk factors and mortality associated with calciphylaxis in end-stage renal disease. Kidney Int 2001 Jul; 60:324-32.

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Ahmed S et al. Calciphylaxis is associated with hyperphosphatemia and increased osteopontin expression by vascular smooth muscle cells. Am J Kidney Dis 2001 Jun; 37:1267-76.

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Llach F. The evolving pattern of calciphylaxis: Therapeutic considerations. Nephrol Dial Transplant 2001 Mar; 16:448-51.

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Riegert-Johnson DL et al. Calciphylaxis associated with cholangiocarcinoma treated with low-molecular-weight heparin and vitamin K. Mayo Clin Proc 2001 Jul; 76:749-52.

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