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Management of refractory cutaneous dermatomyositis in adults

Ruth Ann Vleugels, MD, MPH
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD


Classic dermatomyositis (DM) is an idiopathic inflammatory myopathy that most commonly presents with progressive, symmetric, proximal muscle weakness and a group of characteristic cutaneous findings. The cutaneous manifestations of DM may also develop in the absence of detectable muscle disease and can persist after the successful treatment of DM-associated myositis. (See "Clinical manifestations of dermatomyositis and polymyositis in adults".)

Skin lesions of DM are often resistant to photoprotection and topical therapies alone, necessitating the initiation of systemic therapy. Patients who fail to respond to antimalarials and methotrexate, or who relapse during therapy with these agents, require more aggressive immunosuppressive or immunomodulatory drugs. (See "Initial management of cutaneous dermatomyositis in adults".)

The management of patients with refractory cutaneous DM will be discussed here. The initial treatment of cutaneous DM, as well as the clinical manifestations, diagnosis, and management of the extracutaneous manifestations of DM and juvenile DM, are reviewed elsewhere. (See "Initial management of cutaneous dermatomyositis in adults" and "Clinical manifestations of dermatomyositis and polymyositis in adults" and "Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults" and "Initial treatment of dermatomyositis and polymyositis in adults" and "Treatment of recurrent and resistant dermatomyositis and polymyositis in adults" and "Juvenile dermatomyositis and polymyositis: Epidemiology, pathogenesis, and clinical manifestations" and "Juvenile dermatomyositis and polymyositis: Treatment, complications, and prognosis".)


DM is associated with characteristic cutaneous findings, most notably a pruritic, violaceous to erythematous eruption involving the scalp, face, neck, upper trunk, arms, and dorsal hands (picture 1A-F). Pruritus associated with DM can be severe, leading to significant detrimental effects on patient quality of life. (See "Clinical manifestations of dermatomyositis and polymyositis in adults", section on 'Skin findings'.)

For a subset of patients with skin manifestations of DM, trials of multiple therapies (in combination or sequentially) are required to identify the treatment regimen that produces satisfactory disease control. Because many patients fail to improve adequately with photoprotection, antipruritics, and topical corticosteroids or topical calcineurin inhibitors, the addition of systemic antimalarials or methotrexate is often necessary. Patients who fail to respond to these conventional interventions or who relapse after an initial response have refractory disease and require the initiation of more aggressive therapies. (See "Initial management of cutaneous dermatomyositis in adults", section on 'Treatment'.)

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Literature review current through: Oct 2017. | This topic last updated: Oct 09, 2017.
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