Calciphylaxis in Chronic Renal Failure
Barbara Hahler
Complications of renal failure are being seen more frequently by medical-surgical nurses as these patients are admitted for various problems. Calciphylaxis, a necrotic skin disorder, is a complication of renal failure that may go unrecognized by physicians and nurses. Tissue necrosis may be so extensive that death occurs. It is important for medical-surgical nurses to understand the pathology that occurs during the calciphylactic process.
History
The disease process of calciphylaxis (vascular calcification-cutaneous necrosis syndrome) was first described by Bryant and White in 1898 (Bryant & White, 1898), but the term was first used by Selye in 1962 to describe the sudden, progressive, and widespread calcification of soft tissues. It is a rare, but life-threatening condition that occurs in approximately 1% of patients with end-stage renal disease (Budisavljevic, Cheek, & Ploth, 1996). The time of renal failure diagnosis to the time of symptom onset varies dramatically ranging from 1 week to 7 years. Between 32% to 38% of calciphylaxis patients are recipients of kidney transplants. Women affected outnumber men by almost 3:1 (Angelis, Wong, Myers, & Wong, 1997). Many patients also have diabetes mellitus and/or a history of cardiac disease. Chan, Mahony, Turner, and Posen (1983) reported no pattern of onset of skin ulceration location. Calciphylaxis shows no discrimination toward gender, age, race, or areas of tissue ulceration (Kuzela, Huffer, Conger, Winter, & Hammond, 1977).
Pathology
Various conditions lead to chronic renal failure, which is the inability to filter and therefore eliminate waste products from the blood. Chemical imbalances in phosphorus and calcium occur. Renal phosphate excretion disappears, causing hyperphosphatemia, which leads to calcium release from the bone matrix. Calcium settles along the irregular lining of the arteriole intima. The build-up of calcium narrows the lumen and restricts oxygenated blood from entering the surrounding area. The result is impaired tissue oxygenation. Chemical reactions accompany the decrease in blood flow and this results in further cellular decomposition and necrosis (Selye, 1962). X-rays show calcification of the medium and small arteries. The calcifications may not always be diagnostic as patients with diabetes mellitus frequently have calcified vessels (Duh, Lim, & Clark, 1991). There are no blood tests to diagnose calciphylaxis. Biopsy of the skin lesions reveals subcutaneous tissue calcification and ischemic epidermolysis. A giant cell reaction to the classic calcification in calciphylactic tissue is seen (Fischer & Morris, 1995). The lesions usually begin as a painful mottling of the skin, progressing to a central ulceration with necrosis. Calciphylaxis typically involves the distal extremities, and has been reported to affect the buttocks, lower abdomen, and thighs (Scheinman, Helm, & Fairley, 1991).
Cutaneous calciphylaxis has a grave prognosis, even when aggressively treated via parathyroidectomy. The mortality rate may be as high as 80%. The usual cause of death is septicemia (Whittam, McGibbon, & MacDonald, 1996). Calciphylaxis manifests as erythema, tenderness, and pain. Some patients have reported pain up to I year prior to skin lesion manifestation (Edelstein, Wickman, & Kirby, 1992). Lesions with irregular borders may occur over any part of the trunk or the extremities. Lesions may be as large as 20 cm. Necrosis occurs as tissue ischemia increases. Depth of tissue injury is most commonly limited to the subcutaneous and dermal layers. There may be clear to white exudate from the lesions. Abscesses may develop in deeper tissue layers, leading to sepsis (Duh et al., 1991). Unlike other arterial insufficiency conditions, no large vessel involvement occurs, therefore the absence of peripheral pulses is not present (Saganich, 1996) (see Table 1).
Table 1.
Appearance of Calciphylactic Lesions
* Commonly found on hands, fingers, lower extremities, trunk, and buttocks.
* Painful, tender, firm subcutaneous nodules.
* Lesions may initially appear erythematous or purpuric.
* Nodules become necrotic.
* Necrotic tissue ulcerates.
* Abscesses may develop in deeper tissues.
Treatment
There are few options available for treating calciphylaxis. Treatment is usually supportive. The normalization of serum phosphorus levels via the administration of oral phosphate binders (aluminum hydroxide gel) is one therapeutic goal (Fox, Banowsky, & Cruz, 1983). A low phosphorus diet may also be of benefit. The calcium-phosphorus product should also be lowered by using dialysate with a low-calcium concentration if the patient is hypercalcemic (Duh et al., 1991). Wound infection should be treated with systemic antibiotics, local wound debridement, and incision and drainage of abscesses. Dry gangrene of fingers and toes may be left open to the air and left to autoamputation (Duh et al., 1991).
The definitive surgical treatment for calciphylaxis is parathyroidectomy. Subtotal parathyroidectomy is the surgery of choice, usually removing 3.5 glands. Two-thirds of patients who had surgery survived compared with only one-third who did not have surgery (Hafner et al., 1995).
Conclusion
Calciphylaxis is a devastating disease that frequently has a rapid progression. The cause is unknown, and the treatment is frequently symptomatic. Early recognition of the disease is essential to increase the chance of survival. Patients with renal failure who are receiving dialysis and who complain of painful proximal myopathy should be carefully assessed for developing skin lesions.
References
Angelis, M., Wong, L., Myers, S., & Wong, L. (1997). Calciphylaxis in patients on hemodialysis: A prevalence study. Surgery, 122, 1083-1090.
Bryant, J.H., & White, W.H. (1898). A case of calcification of the arteries and obliterative endarterities associated with hydronephrosis in a child age six months. Guys Hospital Report, 55, 1719.
Budisavljevic, M.N., Cheek, D., & Ploth, D.W. (1996). Calciphylaxis chronic in renal failure. Journal of American Society of Nephrology, 7(7), 978-982.
Chan, Y.L., Mahony, J.F., Turner, J.J., & Posen, S. (1983). The vascular lesions associated with skin necrosis in renal disease. British Journal of Dermatology, 109, 85-95.
Duh, Q., Lim, R., & Clark, O. (1991). Calciphylaxis in secondary hyperparathyroidism. Archives of Surgery 126, 1213-1219.
Edelstein, C.L., Wickman, M.K., & Kirby, PA. (1992). Systemic calciphylaxis presenting as a painful, proximal myopathy. Postgraduate Medicine, 68 209-211.
Fischer, A.H., & Morris, D.J. (1995). Pathogenesis of calciphylaxis: Study of three cases with literature review. Human Pathology, 26, 1055-1064.
Fox, R., Banowsky, L.H., & Cruz, A.B. (1983). Post renal transplant calciphylaxis. Successful treatment with parathyroidectomy. Journal of Urology, 129, 362-363.
Hafner, J., Keusch, G., Wahl, C., Sauter, B., Hurlimann, A., vonWeizsacker, F., Krayenbuhl, M., Biedermann, K., Brunner, U., & Helfenstein, U. (1995). Uremic small-artery disease with medial calcification and intimal hyperplasia (so-called calciphylaxis): A complication of chronic renal failure and benefit from parathyroidectomy. Journal of the American Academy of Dermatology, 33(6), 954-962.
Kuzela, D.C., Huffer, W.E., Conger, J.D., Winter, S.D., & Hammond, W.S. (1977). Soft tissue calcification in chronic dialysis patients. American Journal of Pathology, 86(2), 403-424.
Saganich, B. (1996). Calciphylaxis in renal failure. Wounds, 8(2), 49-52.
Scheinman, P., Helm, K., & Fairley, J. (1991), Acral necrosis in a patient with chronic renal failure. Calciphylaxis. Archives of Dermatology. 127, 248.
Selye, H. (1962). Calciphylaxis. Chicago, IL: University of Chicago Press.
Whittam, L.R., McGibbon, D.H., & MacDonald, D.M. (1996). Proximal cutaneous necrosis in association with chronic renal failure. British Journal of Dermatology, 135, 778-781.
Barbara Hahler, MSN, RN, CS, CETN, is a Clinical Nurse Specialist for Wound, Ostomy, and Continence Nursing, St. Vincent Mercy Medical Center, Toledo, OH.
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