Calciphylaxis in three patients with end-stage renal disease

Abstract

Background: Calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small- and medium-sized vessel calcification seen almost exclusively in patients with end-stage renal disease and hyperparathyroidism. Two patients had bullous lesions preceding their ulcerative lesions, an unusual presentation of this entity. One patient also had penile involvement that, to our knowledge, has not been described previously.

Observations: Three patients, all of whom were being maintained on hemodialysis, developed painful, progressive leg ulcerations. Two patients had elevated parathormone levels, and the third patient did not. All patients had only very modest increases in their calcium x phosphate product.

Conclusions: Calciphylaxis should be included in the differential diagnosis of panniculitis and vasculitis. It is important to diagnose promptly, as early treatment may prevent progression.