Rituximab treatment of refractory skin involvement in anti-TIF1 gamma dermatomyositis

Dermatomyositis is one type among a heterogeneous group of idiopathic inflammatory myopathies. Among these anti-TIF1 gamma dermatomyositis is characterized by specific skin lesions, often severe and refractory to conventional treatments. We report a 58-year-old woman who had fatigue associated with myalgia with proximal and bilateral muscle weakness along with a generalized lilac erythematous rash on the face with Gottron papules on the metacarpophalangeal joints and periungual erythema on both hands. She also exhibited a widespread dark-violaceous-red skin eruption on the whole trunk. She was diagnosed with anti-TIF1 gamma dermatomyositis and received a treatment regimen of topical corticosteroids, hydroxychloroquine, oral corticosteroids, and conventional immunosuppressive drugs (methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide, and immunoglobulins) with no improvement of the skin rash. Therefore, she received rituximab, and three months later, the skin lesions improved magnificently. Rituximab is an efficient and safe option for patients with dermatomyositis-related skin disease refractory to conventional treatments.