A Single-Center Retrospective Analysis of Sweet Syndrome Patients from the Perspective of VEXAS Syndrome Diagnosis

Abstract

Dermatologists play a crucial role in the diagnosis of VEXAS syndrome, given its high incidence of neutrophilic dermatosis and other cutaneous manifestations. We conducted a retrospective investigation to explore the potential diagnosis of VEXAS syndrome in patients previously diagnosed with Sweet syndrome. Our findings revealed that six of the 27 patients who underwent multiple biopsies for refractory skin lesions had histiocytoid Sweet syndrome (HSS), a feature consistent with VEXAS syndrome. No UBA1 mutations were identified in this cohort. Nevertheless, given that HSS can also be the initial manifestation of VEXAS syndrome, early diagnosis through more sensitive genetic testing and appropriate therapeutic intervention is crucial.