Clinicopathological Challenge: Fixed Urticaria‐Like Pink Papules in a Patient With Recurrent Fevers and Arthralgias

A 67-year-old male with a history of macrocytic anemia presented with widespread pink urticarial papules on his trunk and extremities in the setting of intermittent fevers, lymphadenopathy, arthralgias, and night sweats. Repeat biopsies demonstrated mixed inflammatory infiltrate with eosinophils. Multidisciplinary workup revealed a UBA1 p.Met41Thr variant, confirming a diagnosis of Vacuoles E1 Enzyme X-linked Autoinflammatory Somatic Mutations (VEXAS) syndrome manifesting with urticarial dermatitis.