BACKGROUND: Calcific uraemic arteriolopathy (calciphylaxis) is an unusual and 
potentially fatal condition characterised by small-vessel calcification and 
ischaemic skin necrosis. It mainly affects patients with end-stage renal disease 
(ESRD) on haemodialysis, but may rarely occur in the absence of ESRD in 
conditions such as primary hyperparathyroidism, malignancy, alcoholic liver 
disease and connective tissue disease.
METHODS: We reviewed the records of all patients diagnosed with calciphylaxis 
while on renal replacement therapy at Tygerberg Hospital, Cape Town, South 
Africa, between 1990 and 2014, to describe its presentation, course and final 
outcome.
RESULTS: Nineteen patients developed calciphylaxis over this period. Their 
median age was 34 years and 13 (68.4%) were female. Fifteen (78.9%) had received 
a kidney transplant. All patients had painful skin lesions that rapidly 
progressed to infarction. Small-vessel calcification was seen on skin biopsy in 
13 patients. Twelve patients had hyperparathyroidism. Several of the 
transplanted patients had been treated for graft rejection in the year preceding 
the diagnosis. Treatment consisted of good wound care and efforts to normalise 
serum calcium and phosphate levels. Five patients received an urgent 
parathyroidectomy. The outcome was fatal in 17 patients, with sepsis being the 
main cause of death.
CONCLUSIONS: In our patients, calciphylaxis carried a worse prognosis than 
previously reported internationally. It should always be considered in the 
differential diagnosis of painful skin lesions in the dialysis or transplant 
patient.