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.
Neuhaus IM, Connolly MK. An Unusual Presentation of Amyopathic Dermatomyositis Associated With Fatal Interstitial Lung Disease. Arch Dermatol. 2006;142(1):109-116. doi:10.1001/archderm.142.1.113