Calciphylaxis mimicking dermatomyositis: ischemic myopathy complicating renal failure

Among the complications of chronic renal failure is a syndrome of medial calcification of small- to medium-sized arteries associated with ischemic necrosis of the skin and other organ systems, leading to gangrene and a poor prognosis. The syndrome has been reviewed in the renal, dermatologic, and surgical literature under the term calciphylaxis, which describes a postulated pathogenetic mechanism whereby sensitization to an endogenous or exogenous substance (such as parathyroid hormone) predisposes to calcium deposition after exposure to a challenging agent. Myopathy has rarely been reported as the presenting feature, and the syndrome has not been discussed in the neurologic literature.

We report two patients with renal failure and systemic calciphylaxis who presented to our hospital with myopathic complaints and signs suggesting dermatomyositis. We also discuss possible disease mechanisms and treatment.

Because early treatment (including aggressively lowering the calcium and phosphate levels and parathyroidectomy) may improve the outcome, early recognition of the syndrome of calciphylaxis is essential.