VEXAS Syndrome with Vibrio vulnificus Sepsis during Treatment with Tocilizumab.

We report the first case of Vibrio vulnificus sepsis in a patient with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. A 76-year-old man was diagnosed with VEXAS and myelodysplastic syndromes. He was treated with tocilizumab, oral prednisolone, and regular blood transfusions. The patient presented with fever, chills, and vomiting and was subsequently diagnosed with sepsis. A blood culture confirmed V. vulnificus infection, which resolved after tazobactam/piperacillin treatment. V. vulnificus infections can be facilitated by iron overload resulting from frequent blood transfusions. Since patients with VEXAS syndrome often require regular transfusions, clinicians should monitor for this potentially fatal infection.