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

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...

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Bibliographic Details
Published in:Journal of cutaneous pathology 2024-11
Main Authors: Ko, Christine J, Odell, Ian, Gehlhausen, Jeffrey R, Leventhal, Jonathan, McNiff, Jennifer M, Zubek, Amanda
Format: Article
Language:English
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Summary: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.
ISSN:0303-6987
1600-0560
1600-0560
DOI:10.1111/cup.14757