VEXAS Syndrome: Histiocytoid Cells With Feathery Cytoplasm as a Clue to the Diagnosis

Abstract

VEXAS (Vacuoles, E1-ubiquitin activating enzyme UBA1 variant, X-linked, Autoinflammatory, Somatic) syndrome was initially described as having mature neutrophil-predominant infiltrates. More recent reports suggest that infiltrates can be composed of variable cell types. We report three cases of VEXAS syndrome with seven total biopsies having in common histiocytoid cells with feathery cytoplasm; these cells may be a potential clue to the diagnosis.