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Distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome
- Source :
- Experimental Hematology & Oncology, Vol 11, Iss 1, Pp 1-4 (2022)
- Publication Year :
- 2022
- Publisher :
- BMC, 2022.
-
Abstract
- 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.
Details
- Language :
- English
- ISSN :
- 21623619
- Volume :
- 11
- Issue :
- 1
- Database :
- Directory of Open Access Journals
- Journal :
- Experimental Hematology & Oncology
- Publication Type :
- Academic Journal
- Accession number :
- edsdoj.47836798ce40b88cc06377d81f330c
- Document Type :
- article
- Full Text :
- https://doi.org/10.1186/s40164-022-00262-5