Abstract

Dermatomyositis (DM) is an idiopathic inflammatory myopathy that is clinically challenging to diagnose and has a poor prognosis. It is characterized by symmetric proximal muscle weakness, muscle tenderness, dysphagia, characteristic skin rash (heliotrope rash, Gottron's sign), elevated muscle enzyme levels, abnormal electromyography, and muscle biopsy findings. DM with positive anti-MDA5 antibodies is mainly characterized by Gottron's sign, skin ulcers, facial erythema, mechanic's hands, and V-sign. In this case, the patient presented with the rare manifestation of severe necrotic skin ulcers in association with Gottron's sign, prompting us to report this case. A 45-year-old female was admitted to the hospital with systemic joint pain, fatigue, multiple ulcers, and purulent discharge on both hands. Her myositis-specific antibody profile revealed positive anti-MDA5 and anti-SSA/RO52 antibodies. Treatment included a combination of glucocorticoids, immunosuppressants, gastric and liver protection, infection control, and wound care. After two weeks of treatment, the patient showed improvement in symptoms. However, on the 24th day of hospitalization, the wound at the right elbow joint ruptured and became infected, requiring debridement and skin grafting in the appropriate department. There has been limited research and reported cases of dermatomyositis with coexistence of positive anti-MDA5 and anti-SSA/RO52 antibodies combined with severe skin ulcers. Therefore, we present this rare case and emphasize the need for close follow-up on pulmonary involvement and skin ulcer progression, as well as timely implementation of new treatment strategies to actively improve the prognosis.

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