A case of Wong-type dermatomyositis treated with rituximab and IVIG

A 37-year-old woman presented to our rheumatology-dermatology clinic with a rash, muscle weakness and fatigue. She has had prior diagnoses of cutaneous lupus and lichen planus based on skin biopsies. She did not respond to topical steroids, hydroxychloroquine and dapsone. Clinically, she had sharply demarcated photo-distributed erythema over the upper back, chest and upper arms, along with hyperkeratotic follicular papules on bilateral upper arms, shoulders, posterior neck, behind the ears, chest including breasts, abdomen and right buttock. Investigations revealed a high titre ANA, elevated creatinine kinase, aldolase and positive anti-MJ/nuclear matrix protein 2 (NXP-2). A skin biopsy showed findings of connective tissue disease. The diagnosis of Wong-type dermatomyositis was made. She responded to therapy with mycophenolate mofetil, rituximab and IVIG.