DISCUSSION


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.


SUMMARY

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.

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All electronic documents accessed July 15, 2012.


This article originally appeared on Clinical Advisor