| Summary: | Introduction/objectives: The anti-melanoma differentiation-associated gene 5 (Anti-MDA5) antibody is associated with rapidly progressive interstitial lung disease (RP-ILD) and clinically amyopathic dermatomyositis (CADM). The predictors of treatment response would help in classifying the subgroups and decision-making. Here, we aimed to report an observational skin lesion that might be associated with a grave prognosis in six patients with anti-MDA5 antibody-positive ILD. Methods: This case series included 6 anti-MDA5 antibody-positive ILD patients, who were admitted to Songklanagarind Hospital between January 2018 and June 2020. Their medical records were reviewed for clinical phenotypes, laboratory results, imaging studies, treatment, and outcomes. The cutaneous manifestations associated with fatal outcomes were observed and reported. Results: Among 6 patients with anti-MDA5 antibody-positive ILD, 5 patients had CADM and one patient had no skin involvement. Four patients manifested as RP-ILD within a few months. Three deaths occurred despite highly intensive immunosuppressive treatment. All the patients in the dead group exhibited erythematous papules on their auricles and a presence of pulmonary consolidation at lower lung fields was additionally observed. Conclusion: Erythematous auricular papules may be a hallmark of grave prognosis in anti-MDA5 positive CADM with ILD.
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