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Open Access 01-12-2022 | Neutrophilic Dermatosis | Letter to the Editor

Distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome

Authors: Valentin Lacombe, Annaelle Beucher, Geoffrey Urbanski, Yannick Le Corre, Laurane Cottin, Anne Croué, Anne Bouvier

Published in: Experimental Hematology & Oncology | Issue 1/2022

Abstract

VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is an inflammatory disorder with hematological and systemic features. A recent study demonstrated that the dermal infiltrate in neutrophilic dermatosis from VEXAS patients is derived from the pathological UBA1-mutated myeloid clone. Neutrophilic dermatosis is, however, only one of the various skin involvements observed in VEXAS syndrome. We analyzed 10 formalin-fixed paraffin-embedded skin biopsies from genetically confirmed VEXAS syndrome. UBA1 mutation was found in the biopsies related to neutrophilic dermatitis but in none of the other histological patterns (leukocytoclastic vasculitis and septal panniculitis). This could lead to a distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome, which could in turn improve therapeutic outcomes.

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Title: Distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome
Authors: Valentin Lacombe
Annaelle Beucher
Geoffrey Urbanski
Yannick Le Corre
Laurane Cottin
Anne Croué
Anne Bouvier
Publication date: 01-12-2022
Publisher: BioMed Central
Keywords: Neutrophilic Dermatosis
Erythema Nodosum
Vasculitis
Erythema Nodosum
Vasculitis
Leukocytoclastic Vasculitis
Erythema Nodosum
Panniculitis
Leukocytoclastic Vasculitis
Panniculitis
Sweet's Syndrome
VEXAS Syndrome
Autoinflammatory Diseases
Published in: Experimental Hematology & Oncology / Issue 1/2022
Electronic ISSN: 2162-3619
DOI: https://doi.org/10.1186/s40164-022-00262-5

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