Article
UBA1 Variations in Neutrophilic Dermatosis Skin Lesions of Patients With VEXAS Syndrome.
JAMA dermatology - 1 Nov 2021
Zakine Eve, Schell Bérénice, Battistella Maxime, Vignon-Pennamen Marie-Dominique, Chasset François, Mahévas Thibault, Cordoliani Florence, Adès Lionel, Sébert Marie, Delaleu Jérémie, Jachiet Marie, Lepelletier Clémence, Lemaire Pierre, Chauvel Clémentine, Dhouaieb Bedis, Kim Rathana, Cassius Charles, Georgin-Lavialle Sophie, Mekinian Arsène, Bagot Martine, Braun Thorsten, Rousset Laurie, Begon Edouard, de Masson Adèle, Fenaux Pierre, Clappier Emmanuelle, Bouaziz Jean-David
Abstract excerpt
IMPORTANCE: 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. OBJECTIVE: To better describe clinical and...
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