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Article

VEXAS syndrome: A dermatological perspective.

The Australasian journal of dermatology - 1 Nov 2022

Nguyen Jacqueline K, Routledge David, van Der Weyden Carrie, Blombery Piers, Angel Christopher M, Johnson Daryl, Goh Michelle S, Lee Adriene

Abstract excerpt

VEXAS (Vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic mutation) syndrome is a genetically defined disorder identified in 2020, describing patients with inflammatory syndromes associated with haematological dysfunction. It is a severe, treatment-resistant condition, with estimated mortality between 40% and 63%. A wide range of cutaneous manifestations have been described. Here, we report on two...

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PMID: 36197697
DOI: 10.1111/ajd.13932

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