Recalcitrant ulcers associated with SAE-positive dermatomyositis treated with surgery followed by intravenous immunoglobulin

Abstract

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by skeletal muscle abnormalities and characteristic cutaneous lesions, including Gottron's papules and heliotrope rash.DMrelated cutaneous ulcers are uncommon and generally resistant to various treatments. IVIg has been reported as a safe and effective treatment for refractory cutaneous DM. However, there are only a few case reports detailing the use of IVIg in DM related ulcers. Although medical treatments are generally preferred for ulcers unrelated to calcinosis, surgery may be considered for these patients under exceptional circumstances. We report a case of a 41 year-old woman with recalcitrant ulcers associated with DM that were unresponsive to multiple therapies, including topical tacrolimus, topical betamethasone dipropionate, prednisolone, hydroxychloroquine and methotrexate. Due to imminent risk of extensor tendon rupture and the difficulties accessing IVIg within the ideal timeframe, she underwent urgent surgical closure for the ulcer. She subsequently received IVIg postsurgery to expedite healing and prevent Koebnerisation. The surgical repair was successful and all other ulcers healed completely within two weeks after IVIg. Six months after the first dose of IVIg, she was commenced on a three monthly IVIg regime to keep her ulcers in remission. She remained ulcer-free for 18 months since the first dose of IVIg. IVIg should be considered early for patients with recalcitrant DM ulcers. In our experience, surgical intervention is safe and effective, and could be offered to these patients under selected circumstances. We also highlight the possible association between these ulcers and anti- SAE antibody.