Cutaneous Ulcerations in Anti-MDA5 Dermatomyositis
A 48-year-old man presented to the dermatology clinic with painful hand ulcerations. Initial symptoms had begun 6 months earlier and were associated with new-onset shortness of breath on exertion.

Progressively worsening ulcerations on the fingertips and dorsal surface of the hands, elbow and oral ulcerations, and a violaceous periorbital eruption had developed. The patient had no muscle weakness or arthritis.

Computed tomography (CT) of the chest revealed ill-defined nodular opacities that were suggestive of organizing pneumonia. The patient received a diagnosis of anti–melanoma differentiation–associated protein 5 (anti-MDA5) dermatomyositis, which was confirmed on antibody testing. Colonoscopy, CT scans of the abdomen and chest, and prostate-specific antigen testing showed no evidence of cancer.

Anti-MDA5 dermatomyositis is a variant of dermatomyositis associated with interstitial lung disease that can rapidly progress. Characteristic cutaneous findings include ulcerations of Gottron’s papules, oral ulcerations, painful palmar papules, and alopecia.

Immunosuppressive and immunomodulatory therapies (including hydroxychloroquine, systemic glucocorticoids, mycophenolate mofetil, and intravenous immune globulin), as well as therapies to improve blood flow to the hands (aspirin, pentoxifylline, nifedipine, sildenafil, and botulinum toxin), were administered. With this regimen, the patient’s hand ulcers healed within 5 months, and his pulmonary symptoms abated.

Source: https://www.nejm.org/doi/full/10.1056/NEJMicm1816147
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