Childhood-onset hypocomplementaemic urticarial vasculitis in France: phenotypic and genotypic diversity in 10 children
Authors' conclusion
These 10 cases highlight the heterogeneity and potential severity of childhood-onset HUV.
Study context
- Population
- Children with childhood-onset hypocomplementaemic urticarial vasculitis (HUV) in Franceall girls
- Participants
- 10 children
- Exposure
- Systemic HUV syndrome (S-HUVS) vs non-systemic HUVS (NS-HUVS) vs systemic lupus erythematosus (SLE)-associated HUV
- S-HUVSn = 3
- NS-HUVSn = 4
- SLE-associated HUVn = 3
- Outcome
- Clinical and genetic features of childhood-onset HUV
Methodological detailsnationwide, case series
- Nationwide
- Case series
Main finding
As reported in the abstract
- Median age at onset (range 2-14 years)
- 9.5 years
- Median delay onset to diagnosis (range 6-55 months)
- 13 months
- Musculoskeletal features (most common extracutaneous manifestation)
- 9/10
- Pulmonary haemorrhage in S-HUVS
- 2/3
- Glomerulonephritis in S-HUVS
- 2/3
- ANA positive
- 9/10
- Anti-C1q antibodies positive
- 6/10
- ANCA positive
- 5/10
- HCQ used
- 8/10
Observational data: associations, not effects.
Original publication
Childhood-onset hypocomplementaemic urticarial vasculitis in France: phenotypic and genotypic diversity in 10 children. Pediatr Rheumatol Online J. Published 10 March 2026. doi:10.
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