Ko Christine J, Odell Ian, Gehlhausen Jeffrey R, Leventhal Jonathan, McNiff Jennifer M, Zubek Amanda
Department of Dermatology, Yale University, New Haven, Connecticut, USA.
Department of Pathology, Yale University, New Haven, Connecticut, USA.
J Cutan Pathol. 2025 Feb;52(2):108-112. doi: 10.1111/cup.14757. Epub 2024 Nov 28.
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.
VEXAS（空泡、E1泛素激活酶UBA1变体、X连锁、自身炎症性、体细胞）综合征最初被描述为以成熟中性粒细胞为主的浸润。最近的报告表明，浸润可由多种细胞类型组成。我们报告了3例VEXAS综合征患者，共7次活检，其共同特征是存在具有羽毛状细胞质的组织细胞样细胞；这些细胞可能是诊断的潜在线索。
