VEXAS Syndrome: A Comprehensive Review for Dermatologists Part 1: Epidemiology, Pathophysiology, and Clinical Presentations.

Authors

Maria Kaltchenko, Saloni Patel, Anjana Srikumar, Amy DeZern, Marcela Ferrada, Xiao Peng, Jun Kang

Published in

Journal of the American Academy of Dermatology. Aug 17, 2026. Epub Aug 17, 2026.

Abstract

VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is a newly recognized, ubiquitin-activating enzyme E1 (UBA1)-mediated autoinflammatory disorder characterized by treatment-refractory systemic inflammation and diverse cutaneous findings. Dermatologists are uniquely positioned to facilitate early diagnosis because skin involvement occurs frequently and often precedes multi-organ disease. This 2-part continuing medical education series provides an up-to-date overview of VEXAS syndrome. Part 1 reviews epidemiology, pathogenesis, and key clinical and laboratory features, emphasizing dermatologic presentations that should raise suspicion for VEXAS syndrome. Part 2 outlines the diagnostic work-up, including bone marrow findings, definitive UBA1 genetic testing, and management strategies alongside emerging data on prognosis. Together, these articles equip clinicians to recognize VEXAS syndrome, differentiate it from common mimickers, and guide appropriate evaluation and treatment.

PMID:
42607951
Bibliographic data and abstract were imported from PubMed on 18 Aug 2026.

Read full publication at:
Please sign in to see all details.

Stats

1-terrible, 9-excellent. How would you rate this publication? Sign in in to submit your rating.

Post a comment

You need to be signed in to post comments. You can sign in here.

Comments

There are no comments yet.