After years of limited progress, the treatment landscape for dermatomyositis is finally evolving, according to experts speaking at the European Academy of Dermatology and Venereology meeting in Vienna. Victoria Werth of Penn Medicine described the moment as exciting, noting that new therapies could also help patients with paraneoplastic disease. The shift is driven by recent regulatory approvals and a pipeline of ongoing clinical trials.
For now, first-line management remains unchanged: broad-spectrum sunscreens with SPF 70 or higher, topical steroids, and antimalarials such as hydroxychloroquine. Werth said antimalarials help about 20% of her patients avoid escalation to stronger drugs. When second-line treatment is needed, immunosuppressives like methotrexate and mycophenolate mofetil are common, but for paraneoplastic patients she prefers IVIG to avoid additional immunosuppression. Oral steroids are used but should be tapered quickly, and JAK inhibitors are generally avoided in patients with cancer histories due to uncertain malignancy risk.
The first FDA-approved therapy for dermatomyositis, IVIG, was studied mainly in muscle-predominant disease, leaving skin-predominant patients with fewer options. That changed with the approval of brepocitinib (Lisraya), an oral TYK2/JAK1 inhibitor, for skin lesions. A phase III trial showed significant and durable improvement, with many patients reaching near-clear skin. Looking ahead, anifrolumab, dazukibart, and efgartigimod have all shown encouraging results in trials, though efgartigimod's dermatomyositis subgroup did not reach statistical significance despite being deemed clinically meaningful.