Article
Clinical and histological features of histiocytoid Sweet syndrome associated with VEXAS syndrome.
Clinical and experimental dermatology - 19 Jul 2024
Lecoeuvre Hortense, Le Gall François, Le Naoures Cécile, Vignon-Pennamen Marie-Dominique, Lamaison Claire, Kammerer-Jacquet Solène-Florence, Lescoat Alain, Oger Emmanuel, Pastoret Cédric, Dupuy Alain
Abstract excerpt
BACKGROUND: VEXAS (Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is caused by acquired somatic mutations in UBA1. Sweet-syndrome-like skin disorders [and especially histiocytoid Sweet syndrome (HSS)] may be associated with VEXAS syndrome. OBJECTIVES: To characterize the clinical and histopathological features of HSS in patients with VEXAS syndrome. METHODS: Skin biopsies with a histological...
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