Distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome.
Autoinflammatory diseases
Clonal hematopoiesis
Mutation
Sweet syndrome
Vasculitis
Journal
Experimental hematology & oncology
ISSN: 2162-3619
Titre abrégé: Exp Hematol Oncol
Pays: England
ID NLM: 101590676
Informations de publication
Date de publication:
16 Feb 2022
16 Feb 2022
Historique:
received:
07
12
2021
accepted:
06
02
2022
entrez:
17
2
2022
pubmed:
18
2
2022
medline:
18
2
2022
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 35172893
doi: 10.1186/s40164-022-00262-5
pii: 10.1186/s40164-022-00262-5
pmc: PMC8848791
doi:
Types de publication
Letter
Langues
eng
Pagination
6Informations de copyright
© 2022. The Author(s).
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