Hortense Lecoeuvre, Franҫois Le Gall, Cécile Le Naoures, Marie-Dominique Vignon-Pennamen, Claire Lamaison, Solène-Florence Kammerer-Jacquet, Alain Lescoat, Emmanuel Oger, Cédric Pastoret, Alain Dupuy
BACKGROUND: "Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic" (VEXAS) syndrome is caused by acquired somatic mutations in the ubiquitin-activating enzyme 1 (UBA1) gene. Sweet-syndrome-like skin disorders (and especially histiocytoid Sweet syndrome (HSS)) may be associated with VEXAS syndrome. OBJECTIVE: To characterize the clinical and histopathological features of HSS in patients with VEXAS syndrome. METHODS: The skin biopsies with a histological diagnosis of HSS had been collected at Rennes University Medical Center (Rennes, France) between October 2011 and January 2022...
February 16, 2024: Clinical and Experimental Dermatology