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A 47‐year‐old woman with mixed connective tissue disease was treated with abatacept. After 2 months, a 3‐cm depression with atrophied surface was observed on her back, which had histopathological consistent with the symptoms of localized scleroderma. Although some cases of paradoxical reaction or cutaneous adverse event have been reported from abatacept, no localized scleroderma has been reported, suggesting this to be a unique case.

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