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What is known and objective Kaposi's sarcoma ( KS) is a malignant neoplasm caused by HHV-8, a pathogen that leads to endothelial cell transformation when host defences are weakened. Case description Here we report the first case of KS during treatment with abatacept, a biologic agent targeting T-cell costimulation. The patient was a 64-year-old female with rheumatoid arthritis who developed multiple firm, purple-reddish nodules on the dorsal aspect of the right hand. Histological examination confirmed KS. What is new and conclusion Although a direct causal relationship between KS development and abatacept treatment cannot be proved, we hypothesize a role for costimulation blockade. [ABSTRACT FROM AUTHOR]