Article
VEXAS-Syndrome, a newly described autoinflammatory systemic disease with dermatologic manifestations.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG - 1 Dec 2023
Baur Vera, Stoevesandt Johanna, Hueber Axel, Hüffmeier Ulrike, Kneitz Hermann, Morbach Henner, Schultz Erwin, Goebeler Matthias
Abstract excerpt
VEXAS syndrome is a recently identified autoinflammatory systemic disease caused by an acquired somatic mutation of the X-linked UBA1 gene, the key enzyme of the first step of ubiquitylation. The acronym VEXAS stands for the characteristics Vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic. The disease occurs in advanced adulthood preferentially in men and is characterized by hematological,...
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