皮疹伴发热、贫血为表现的VEXAS综合征1例

doi:10.35541/cjd.20220676

中华皮肤科杂志 ›› 2025, e20220676.doi: 10.35541/cjd.20220676

皮疹伴发热、贫血为表现的VEXAS综合征1例

刘子琪¹ 林尽染¹ 王倩² 陈连军¹ 陈虎艳¹ 乐艳¹ 张悦¹ 王振华² 朱萍² 丁天凌² 黄琼¹

¹复旦大学附属华山医院皮肤科，上海 200032；²复旦大学附属华山医院血液科，上海 200032

收稿日期:2022-09-21 修回日期:2024-04-11 发布日期:2025-09-22
通讯作者: 黄琼 E-mail:blueqiong1972@163.com
基金资助:
上海市青年科技英才扬帆计划（21YF1404400）

A case of VEXAS syndrome presented with skin lesions, fever and anemia

Liu Ziqi¹, Lin Jinran¹, Wang Qian², Chen Lianjun¹, Chen Huyan¹, Le Yan¹, Zhang Yue¹, Wang Zhenhua², Zhu Ping², Ding Tianling², Huang Qiong¹

¹Department of Dermatology, Huashan hospital, Fudan University, Shanghai 200032, China; ²Department of Hematology, Huashan hospital, Fudan University, Shanghai 200032, China

Received:2022-09-21 Revised:2024-04-11 Published:2025-09-22
Contact: Huang Qiong E-mail:blueqiong1972@163.com
Supported by:
Shanghai Sailing Program（21YF1404400）

摘要/Abstract

摘要： 患者男，57岁，因反复躯干、双上肢皮疹伴发热5年于2022年6月就诊。患者5年前无明显诱因躯干、双上肢出现红色皮疹，伴反复发热，最高41 ℃，发热时伴有关节痛及淋巴结肿大。外院拟“湿疹样皮炎”予口服泼尼松15 mg/d，用药后皮疹及热度可退，但泼尼松减量则症状反复……

刘子琪林尽染王倩陈连军陈虎艳乐艳张悦王振华朱萍丁天凌黄琼 . 皮疹伴发热、贫血为表现的VEXAS综合征1例[J]. 中华皮肤科杂志, 2025,e20220676. doi:10.35541/cjd.20220676

Liu Ziqi, Lin Jinran, Wang Qian, Chen Lianjun, Chen Huyan, Le Yan, Zhang Yue, Wang Zhenhua, Zhu Ping, Ding Tianling, Huang Qiong. A case of VEXAS syndrome presented with skin lesions, fever and anemia[J]. Chinese Journal of Dermatology,2025,e20220676. doi:10.35541/cjd.20220676

参考文献 8

［1］	Beck DB, Ferrada MA, Sikora KA, et al. Somatic mutations in UBA1 and severe adult⁃onset autoinflammatory disease［J］. N Engl J Med, 2020,383（27）:2628⁃2638. doi: 10.1056/NEJMoa 2026834.
［2］	Grayson PC, Patel BA, Young NS. VEXAS syndrome［J］. Blood, 2021,137（26）:3591⁃3594. doi: 10.1182/blood.2021011455.
［3］	Diarra A, Duployez N, Terriou L. Mutant UBA1 and severe adult⁃onset autoinflammatory disease［J］. N Engl J Med, 2021,384（22）:2163⁃2164. doi: 10.1056/NEJMc2102124.
［4］	Georgin⁃Lavialle S, Terrier B, Guedon AF, et al. Further characterization of clinical and laboratory features in VEXAS syndrome: large⁃scale analysis of a multicentre case series of 116 French patients［J］. Br J Dermatol, 2022,186（3）:564⁃574. doi: 10.1111/bjd.20805.
［5］	Sterling D, Duncan ME, Philippidou M, et al. VEXAS syndrome （vacuoles, E1 enzyme, X⁃linked, autoinflammatory, somatic） for the dermatologist［J］. J Am Acad Dermatol, 2023,89（6）:1209⁃1214. doi: 10.1016/j.jaad.2022.01.042.
［6］	Zakine E, Schell B, Battistella M, et al. UBA1 variations in neutrophilic dermatosis skin lesions of patients with VEXAS syndrome［J］. JAMA Dermatol, 2021,157（11）:1349⁃1354. doi: 10.1001/jamadermatol.2021.3344.
［7］	Alegría⁃Landa V, Rodríguez⁃Pinilla SM, Santos⁃Briz A, et al. Clinicopathologic, immunohistochemical, and molecular features of histiocytoid sweet syndrome［J］. JAMA Dermatol, 2017,153（7）:651⁃659. doi: 10.1001/jamadermatol.2016.6092.
［8］	Ferrada MA, Savic S, Cardona DO, et al. Translation of cytoplasmic UBA1 contributes to VEXAS syndrome pathogenesis［J］. Blood, 2022,140（13）:1496⁃1506. doi: 10.1182/blood.2022 016985.

皮疹伴发热、贫血为表现的VEXAS综合征1例

A case of VEXAS syndrome presented with skin lesions, fever and anemia

PDF

PDF (Mobile)

可视化

摘要/Abstract

引用本文

使用本文

参考文献 8

相关文章 0

Metrics

本文评价

推荐阅读 10