Background. Calciphylaxis is a rare, painful, life-threatening problem of c
utaneous necrosis and refractory healing in patients with uremia and second
ary hyperparathyroidism. The pathogenesis involves abnormalities in calcium
and phosphorus metabolism and acute deposition of calcium in tissues.
Method. The clinical course of 16 patients who were diagnosed with calciphy
laxis at our institution from 1994 through 1998 was reviewed.
Results. Fourteen female patients and 2 male patients had chronic renal dis
ease, secondary hyperparathyroidism, and characteristic skin necrosis (mean
age, 56 years, range, 39-70 years). All patients underwent intensive medic
al therapy, including ongoing hemodialysis (n = 16 patients), parathyroidec
tomy (n = 7 patients), and debridement of cutaneous lesions (n = 8 patients
). Mean serum values in surgical and nonsurgical patients were significantl
y different for phosphorus, calcium-phosphorus product, and parathormone le
vels. Median survival was 9.4 months; 15 patients (93%) have died. The medi
an survival time for parathyroidectomy versus nonparathyroidectomy was 14.8
and 6.3 months (P =.22), for skin debridement versus nondebridement was 14
.1 and 6.1 months (P =.08), and for diabetic versus nondiabetic patients wa
s 6.5 and 13.9 months (P =.11).
Conclusions. Calciphylaxis has a female preponderance, with a dismal progno
sis. A multidisciplinary approach that uses frequent hemodialysis to normal
ize calcium and phosphorus levels and local debridement of skin lesions see
ms prudent. Parathyroidectomy cannot be recommended routinely in all patien
ts, unless severe hyperparathyroidism mandates intervention.