VEXAS syndrome as a cause for multifocal, relapsing head and neck inflammation

Abstract

VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a novel autoinflammatory syndrome. We describe a case of VEXAS syndrome with upper airway and oral cavity involvement which are not well described in the literature.

Keywords: Ludwigs angina; UBA1 mutation; VEXAS syndrome; autoinflammation; periorbital cellulitis; venous thromboembolism.