Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) typically presents mild or absent muscle involvement and unique skin lesions such as cutaneous ulceration and palmar papules reflecting vasculopathy, commonly associated with rapidly progressive interstitial lung disease (RP-ILD), causing fatal outcomes (1). This evidence concerns the gene IFIH1 and retinitis pigmentosa 1.