VEXAS Syndrome-Diagnostic Clues for the Dermatologist and Gaps in Our Current Understanding: A Narrative Review

Lowell T Nicholson; Edward W Cowen; David Beck; Marcela Ferrada; Lauren M Madigan

doi:10.1016/j.xjidi.2023.100242

VEXAS Syndrome-Diagnostic Clues for the Dermatologist and Gaps in Our Current Understanding: A Narrative Review

JID Innov. 2023 Oct 29;4(1):100242. doi: 10.1016/j.xjidi.2023.100242. eCollection 2024 Jan.

Authors

Lowell T Nicholson¹, Edward W Cowen², David Beck³, Marcela Ferrada⁴, Lauren M Madigan¹

Affiliations

¹ Department of Dermatology, University of Utah, Salt Lake City, Utah, USA.
² Dermatology Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institute of Health, Bethesda, Maryland, USA.
³ Department of Medicine, New York University Grossman School of Medicine, New York City, New York, USA.
⁴ Rheumatology Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institute of Health, Bethesda, Maryland, USA.

Abstract

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome is a newly recognized, acquired autoinflammatory disorder with broad systemic implications and a poor global prognosis. Because cutaneous lesions are present in the majority of those affected, it is necessary that dermatologists are equipped to recognize this important disease. Through identification, there is a greater opportunity for disease stratification, surveillance for systemic involvement, and selection of the best available therapies. As our understanding of this disease develops, dermatologists should also play a role in addressing the knowledge gaps that exist.

Keywords: Autoinflammatory; Genetics; Hematologic malignancy; Neutrophilic dermatosis; VEXAS.

Publication types

Review