Background: Acquired factor VIII inhibitor is an antibody formed against FVIII and seen in patients without FVIII replacement therapy. This entity has been associated with both autoimmune disease and infections. Case Study: We report the case of a 65-year-old man with dermatomyositis who presented with a bleeding episode following venipuncture and hematuria. His coagulation parameters were followed as well as the inhibitor level. His acquired FVIII inhibitor was managed successfully with rituximab. Conclusion: Our patient’s bleeding episodes resolved and the inhibitor level was undetectable after 12 months. This is the first case of acquired FVIII inhibitor in association with dermatomyositis.

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