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Management Considerations: Refractory Skin Rash and Calcinosis

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Managing Myositis

Abstract

Cutaneous manifestations of dermatomyositis can be severe and debilitating due to intense itching, burning pain, ulceration, and inflammatory pink to violaceous discoloration involving the skin, particularly the scalp, face, chest, and hands. Control of the refractory cutaneous dermatomyositis is challenging and usually requires a combination of systemic immunomodulators and/or immunosuppressants to achieve partial or complete remission. In selecting the therapeutic agents to control cutaneous dermatomyositis while minimizing their toxicities, it is important to consider each patient’s other organ-specific manifestations of dermatomyositis such as myositis, interstitial lung disease, arthritis, and malignancy in addition to the patient’s other health conditions and goals of treatment. Myositis-specific autoantibody may indirectly guide management as these autoantibodies may predict risk of other organ involvement such as interstitial lung disease in patients with anti-MDA5 and anti-synthetase autoantibodies and thereby influence the systemic medications chosen for treatment. Although early treatment of cutaneous dermatomyositis may prevent cutaneous damage such as hyperpigmentation, atrophy, calcinosis, and scarring from ulceration, it is currently unclear if treatment of cutaneous disease impacts involvement of other organs. Treatment of cutaneous calcinosis remains a formidable task. While the currently available medical therapies may have modest effects in reducing the burden and/or symptoms associated with calcinosis, surgical excision continues to be the single most effective treatment.

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Correspondence to David F. Fiorentino .

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Lewis, M.A., Fiorentino, D.F. (2020). Management Considerations: Refractory Skin Rash and Calcinosis. In: Aggarwal, R., Oddis, C. (eds) Managing Myositis. Springer, Cham. https://doi.org/10.1007/978-3-030-15820-0_31

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