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Article

Childhood-onset hypocomplementaemic urticarial vasculitis in France: phenotypic and genotypic diversity in 10 children.

Pediatric rheumatology online journal - 10 Mar 2026

Bianchi Chloe, Melki Isabelle, Sisirak Vanja, Loizon Severine, Broly Martin, Boursier Guilaine, Tusseau Maud, Laurent Audrey, Belot Alexandre, Eitenschenck Laurence, Guigonis Vincent, Jeziorski Eric, Leguevaques Damia, Richer Olivier, Pillet Pascal, Granel Jérôme

Abstract excerpt

OBJECTIVES: Hypocomplementaemic urticarial vasculitis (HUV) is a rare small-vessel vasculitis characterized by the combination of urticarial vasculitis and hypocomplementaemia. HUV may occur in isolation or be associated with systemic diseases such as systemic lupus erythematosus (SLE). Recently, loss-of-function variants in DNASE1L3 have been reported to cause familial forms of HUV. We aimed to describe the...

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Age of Onset
Child
Child, Preschool
Endodeoxyribonucleases

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Lupus Erythematosus, Systemic

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Open in PubMed Open full text

Identifiers

PMID: 41807955
DOI: 10.1186/s12969-026-01203-w
PMCID: PMC13067430

Genes

DNASE1L3
C2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

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