Clinical Images: Cutaneous ulcerations in anti‐MDA‐5 dermatomyositis

The patient, a 41‐year‐old woman, presented to the clinic with skin ulcers. She complained of proximal muscle weakness with difficulty ambulating, diffuse joint pain, dysphagia, shortness of breath, and diffuse rash and ulcers for five months that have been worsening over time. Skin examination was notable for an erythematous scaly scalp, violaceous patches surrounding bilateral eyes with significant periorbital swelling, violaceous patches on lateral thighs consistent with Holster sign, (A) painful ulcers on the dorsum of the hands, (B) elbows with overlying crust, posterior shoulders, and left buttock. She also had (C) ulcerative macules located on the flexor aspects of the fingers. The patient was admitted to the hospital for further evaluation and management. Laboratory work‐up came back positive for anti‐MDA‐5 antibody with elevated creatine kinase, aldolase, and ferritin levels. Imaging of the chest was consistent with interstitial lung disease. Magnetic resonance imaging of her right lower extremity showed edema in the distal vastus intermedius, lateralis muscles, and external obturator muscles. The patient was treated with systemic and topical steroids, intravenous immunoglobulin, cyclophosphamide, and tacrolimus. Her symptoms improved with residual scarring and hyperpigmentation at the site of skin ulcers and complete resolution of dyspnea, dysphagia, and muscle weakness. Repeat high‐resolution computed tomography of the chest showed interval improvement in the ground glass and interstitial reticular opacities. Anti‐MDA‐5 dermatomyositis patients often have characteristic cutaneous manifestations that can help differentiate them from other dermatomyositis subtypes. ¹ They are more likely to have skin or oral ulcers. ¹ Patients with ulcerative Gottron papules are more likely to test positive for MDA‐5 antibody and are at great risk for developing interstitial lung disease. ¹

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