Sleeve Sign and Inverse Gottron’s Papules in Anti-MDA5 Dermatomyositis
A 46-year-old woman presented to the dermatology clinic with a 1-month history of shortness of breath, dry cough, and painful rash on the upper arms. She reported no myalgias or weakness. Her oxygen saturation was 94% while she was breathing ambient air. On physical examination, fine crackles could be heard at the lung bases. Erythematous plaques and patches were seen on the lateral aspects of the upper arms (Panel A), and erythematous patches, papules, and pustules were seen on the ventral surfaces of the hands (Panel B). These findings — known as “sleeve sign” and “inverse Gottron’s papules,” respectively — are rare cutaneous findings associated with dermatomyositis. Laboratory testing showed strong positivity for anti–melanoma differentiation–associated protein 5 (anti-MDA5) antibody and a mildly elevated creatine kinase level. Three weeks after the initial presentation, the patient was admitted to the hospital for progressive dyspnea. High-resolution computed tomography of the chest showed diffuse reticular and nodular opacities. Pulmonary-function tests revealed a restrictive ventilatory defect with decreased diffusion capacity. Whole-body positron-emission tomography showed no occult cancer. A final diagnosis of anti-MDA5 hypomyopathic dermatomyositis with rapidly progressive interstitial lung disease was made. Despite the use of aggressive immunosuppressive therapy, the patient died from respiratory failure 2 months after her initial presentation.