UBA1 Variations in Neutrophilic Dermatosis Skin Lesions of Patients With VEXAS Syndrome | Zendy

Ève Zakine | Zendy; Bérénice Schell | Zendy; Maxime Battistella | Zendy; MarieDominique VigPennamen | Zendy; François Chasset | Zendy; T. Mahévas | Zendy; F. Cordoliani | Zendy; Lionel Adès | Zendy; Marie Sébert | Zendy; J. Delaleu | Zendy; M. Jachiet | Zendy; C. Lepelletier | Zendy; Pierre Lemaire | Zendy; Clémentine Chauvel | Zendy; Bedis Dhouaieb | Zendy; Rathana Kim | Zendy; C. Cassius | Zendy; Sophie GeorginLavialle | Zendy; A. Mékinian | Zendy; Martine Bagot | Zendy; Thorsten Braun | Zendy; L. Rousset | Zendy; É. Bégon | Zendy; Adèle de Masson | Zendy; Pierre Fenaux | Zendy; Emmanuelle Clappier | Zendy; JeanDavid Bouaziz | Zendy

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UBA1 Variations in Neutrophilic Dermatosis Skin Lesions of Patients With VEXAS Syndrome

Author(s) -

Ève Zakine,

Bérénice Schell,

Maxime Battistella,

MarieDominique VigPennamen,

François Chasset,

T. Mahévas,

F. Cordoliani,

Lionel Adès,

Marie Sébert,

J. Delaleu,

M. Jachiet,

C. Lepelletier,

Pierre Lemaire,

Clémentine Chauvel,

Bedis Dhouaieb,

Rathana Kim,

C. Cassius,

Sophie GeorginLavialle,

A. Mékinian,

Martine Bagot,

Thorsten Braun,

L. Rousset,

É. Bégon,

Adèle de Masson,

Pierre Fenaux,

Emmanuelle Clappier,

JeanDavid Bouaziz

Publication year - 2021

Publication title -

jama dermatology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.128

H-Index - 166

eISSN - 2168-6084

pISSN - 2168-6068

DOI - 10.1001/jamadermatol.2021.3344

Subject(s) - medicine , pathology , bone marrow , myeloid , dermatology

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a recently described severe adult-onset autoinflammatory disease that is associated with myeloid lineage-restricted ubiquitin-activating enzyme 1 (UBA1) somatic variations that primarily affect the skin (Sweet syndrome), cartilage, and bone marrow. Skin symptoms have been poorly described.

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