Management of cutaneous dermatomyositis: current therapeutic options

Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations and proximal muscle weakness. Patients with this condition frequently require treatment for muscular, systemic, and cutaneous involvement; however, the cutaneous symptoms often cause significant morbidity and frustration to patients. The skin lesions of dermatomyositis often persist or recur after treatment of myositis, and there are subsets of patients who have significant cutaneous disease with little or no muscle involvement. Only anecdotal evidence is available for cutaneous treatment, and the approach to therapy is guided by disease severity and refractoriness. First-line therapy for localized disease begins with topical agents such as antipruritics and/or corticosteroids, while widespread disease often requires initial systemic therapy. Antimalarials are generally used for significant skin disease, followed by other anti-inflammatory agents, systemic corticosteroids, corticosteroid-sparing immunosuppressants and, recently, biologics and intravenous immunoglobulin. While there is a recognized absence of randomized prospective clinical trials examining the optimal monotherapy and combination treatment for the cutaneous manifestations of dermatomyositis, we have tried to provide a review of the literature and a systematic approach to dealing with these often refractory and debilitating symptoms.