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Calciphylaxis mimicking dermatomyositis

1998; Lippincott Williams & Wilkins; Volume: 51; Issue: 6 Linguagem: Inglês

10.1212/wnl.51.6.1634

1526-632X

Kevin M. Flanigan, Mark B. Bromberg, M. M. Gregory, J. Richard Baringer, Charles Ray Jones, Theresa A. Nester, Edward C. Klatt, Jeannette J. Townsend,

Inflammatory Myopathies and Dermatomyositis

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, 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. Methods: 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. Conclusions: 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.