Vexas Syndrome: A Multisystem Challenge with Sweet Syndrome and Myelodysplastic Syndrome.

Introduction: VEXAS (vacuoles, E1 enzyme, x-linked, autoinflammatory, somatic) syndrome, is a recently described autoinflammatory condition caused by Case description: We present a case of a 43-year-old Arab male with a history of MDS. He presented with painful, non-pruritic, non-haemorrhagic skin lesions. The patient also reported having fever and chills, partially relieved by antipyretics. Bone marrow aspiration was done and revealed a 'dry tap' which was inadequate for evaluation. Immunophenotyping of bone marrow biopsy was consistent with MDS. A skin biopsy of the lesions was performed and confirmed sweet syndrome. The patient was treated with methylprednisolone 500 mg daily for five days, with a significant improvement in clinical symptoms. After stabilisation, treatment for MDS was started with 200 mg IV azacitidine daily for seven days. Conclusion: Given the patient's age, gender, MDS diagnosis and atypical presentation of sweet syndrome, VEXAS syndrome is a strong possibility. Further investigation, including genetic testing for a LEARNING POINTS: Features such as steroid-dependent skin lesions, cytopenias and MDS raise suspicion for VEXAS syndrome - a systemic autoinflammatory disorder linked to