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Dermatomyositis (DM) is an autoimmune vasculopathy that affects the skin and proximal skeletal muscles. In DM, muscle involvement is present at the onset in 50% of cases and ultimately develops in 90% of cases.¹ However, a small subset of patients develop the characteristic skin lesions in the absence of muscle involvement. This condition has been termed amyopathic dermatomyositis (ADM).² The natural history of ADM is considered more benign than DM, and aggressive treatment is not warranted.³ Nonetheless, rarely a patient with ADM can experience severe systemic involvement, including interstitial lung disease.^4-8 We report a case of ADM in which the diagnosis was missed and the patient ultimately died of interstitial lung disease.

Report of a Case

A 39-year-old man presented with an 18-month history of bilateral periorbital swelling and a unilateral heliotrope rash over the right eyelid (Figure). He developed severe arthralgias and myalgias but maintained normal muscle strength, which was documented . . . [Full Text of this Article]

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AUTHOR INFORMATION
Isaac M. Neuhaus, MD; M. Kari Connolly, MD

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