VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic; OMIM #301054) syndrome is an autoinflammatory condition first described in November 2020.1 It is characterized by vacuoles in myeloid and erythroid precursor cells on bone marrow biopsy and caused by a monogenic somatic mutation in the ubiquitin-like modifier activating enzyme 1 (UBA1) gene.1,2.