Article
VEXAS syndrome with eosinophilia and pathologically mimicking histiocytosis: a case report.
Modern rheumatology case reports - 25 Jul 2025
Murai Yasuhisa, Watanabe Rina, Tsurumoto Tatsuya, Kurotaki Hidekachi, Tachita Takuto, Takiyoshi Noriko, Nagaki Takahiko, Kurosaka Naruki, Yanagida Ren, Kikuchi Ryoichi, Takasugi Kaori, Zaimoku Yoshitaka, Amenomori Kyoko, Ota Shinji, Hasui Keisuke, Yamaguchi Satoko, Hiraga Hiroto, Kanazawa Hiroshi, Sakuraba Hirotake
Abstract excerpt
Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome is a recently identified autoinflammatory disorder caused by somatic mutations in the UBA1 gene. This report describes the case of a 54-year-old Japanese man with VEXAS syndrome exhibiting atypical features of eosinophilia and histiocytoid changes that mimic histiocytosis. Initially, the patient presented with recurrent fever, eosinophilia,...
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