Calciphylaxis in secondary hyperparathyroidism. Diagnosis and parathyroidectomy
Q. Y. Duh, R. C. Lim and O. H. Clark
Surgical Service, Veterans Affairs Medical Center, San Francisco, CA 94121.
Calciphylaxis is a rare, severe complication of secondary
hyperparathyroidism. Patients present with painful, violaceous, mottled
skin lesions of the upper and lower extremities, which become necrotic and
produce nonhealing ulcers. Gangrene of fingers and toes frequently requires
amputation, produces nonhealing wounds, and can lead to sepsis and death.
We reviewed the clinical course of five patients with calciphylaxis treated
in our institution. The three men and two women (aged 47 to 72 years) had
secondary hyperparathyroidism from chronic renal failure. All patients had
severe pruritus, painful ulcers, and severe hyperphosphatemia with elevated
serum calcium-phosphate product (greater than 12 mmol2/L2), but the serum
parathyroid hormone levels were only moderately elevated. Most patients had
medical calcification of medium and small blood vessels, and some had
soft-tissue calcification visible on roentgenography. Treatment consisted
of local wound care, antibiotics, phosphate-binding agents, and
parathyroidectomy. Two patients died of uncontrollable sepsis. The three
survivors had dramatic improvement of pain and ulcers after
parathyroidectomy. Calciphylaxis is a limb- and life-threatening
complication of secondary hyperparathyroidism. Diagnosis can be made by
recognizing the characteristic painful skin lesions, ulcers, and gangrene
of the digits, and patients should be treated with subtotal
parathyroidectomy.
