Calciphylaxis mimicking dermatomyositis - Ischemic myopathy complicating renal failure

Background: 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, dermatol ogic, 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 calciu m deposition after exposure to a challenging agent. Myopathy has rarely bee n reported as the presenting feature, and the syndrome has not been discuss ed in the neurologic literature. Methods: We report two patients with renal failure and systemic calciphylaxis who presented to our hospital with myop athic complaints and signs suggesting dermatomyositis. We also discuss poss ible disease mechanisms and treatment. Conclusions: Because early treatment (including aggressively lowering the calcium and phosphate levels and para thyroidectomy) may improve the outcome, early recognition of the syndrome o f calciphylaxis is essential.