A “Leopard Man” Aspect on 18F-FDG PET/CT Revealing a VEXAS Syndrome
Alexandre Fagart, T. Quéméneur, Guillaume Collet, F. Demailly, Clémentine Rousselin
Abstract
ABSTRACT: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset autoinflammatory disease caused by somatic UBA1 mutations first described in 2020. Most of these patients met clinical criteria for an inflammatory syndrome (relapsing polychondritis, Sweet syndrome, polyarteritis nodosa, or giant-cell arteritis) or a hematologic condition (myelodysplastic syndrome or multiple myeloma) or both. We described here an FDG PET/CT "leopard man" appearance, with abnormal marrow recruitment the findings, in a 70-year-old man diagnosed with a VEXAS syndrome.
Topics & Concepts
MedicineRelapsing polychondritisPolyarteritis nodosaPathologyMultiple myelomaGiant cell arteritisDiseaseDermatologyVasculitisInternal medicineOtitis Media and Relapsing PolychondritisVascular Anomalies and TreatmentsIgG4-Related and Inflammatory Diseases