Abstract
Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma with a reported incidence of 4.1 per million person-years. Despite its local invasiveness, it rarely metastasizes (5% of cases). Fibrosarcomatous change in DFSP is a form of tumor progression that carries an increased risk of metastases. We reported a case of 45-year-old patient treated with adalimumab lasting 7 years for Crohn's disease who developed dermatofibrosarcoma protuberans with fibromatous changes. Adalimumab therapy was stopped before surgery, and ustekinumab was introduced 6 months after.