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1
A 76-year-old woman with anti-NXP-2-positive dermatomyositis had progressive extremity erythema, proximal weakness, dysphagia, and dysphonia.
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2
Examination showed bilateral erythematous lesions on the lateral upper arms and erythematous-violaceous lesions on the lateral thighs.
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3
Other findings included posterior neck, forehead, and dorsal hand erythema, dilated nailfold capillaries, elevated creatine kinase, and muscle MRI abnormalities.
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4
The sleeve sign affects the lateral upper arms, and the holster sign affects the lateral hips and proximal thighs; both are typically bilateral and symmetric.
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5
Neither sign is pathognomonic, but both are characteristic of dermatomyositis; the holster sign occurs in 40% to 51% of adults, while the sleeve sign is uncommon.