Abstract: A 72-year-old female presented with skin rashes and pruritus on the face and neck for 16 years, and skin lesions got worsened after sun exposure. The myositis specific autoantibodies panel showed a positive anti-TIF1-γ antibody. Skin biopsy taken from the forehead revealed histopathologic changes of hyperkeratosis, focal parakeratosis and interface dermatitis. Based on the classic manifestations of dermatomyositis and the presence of pityriasis rubra pilaris-like eruptions on the forehead, the diagnosis of Wong-type amyopathic dermatomyositis was made. A treatment with oral acitretin and Haitang Heji was initiated, combined with topical pimecrolimus cream and the patient responded well to the therapy.

Key words: dermatomyositis, pityriasis rubra pilaris