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Case Report: Hypocomplementemic urticarial vasculitis syndrome in a pediatric patient with complement factor 1
Sallie Lin1, Dina Kafisheh1,2, Melissa E Elder1,2
1Department of Pediatrics, University of Florida, Gainesville, FL, United States.
Frontiers in Pediatrics
|October 8, 2024
Summary
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a severe condition. This case study presents a rare co-occurrence of HUVS and complement factor 1 (CF1) deficiency in an adolescent.
Area of Science:
- Immunology
- Rheumatology
- Genetics
Background:
- Urticarial vasculitis (UV) involves immune complex deposition and complement activation.
- Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a severe UV subtype with specific clinical and serological features.
Observation:
- An adolescent patient presented with invasive pneumococcal infection.
- The patient was subsequently diagnosed with both HUVS and complement factor 1 (CF1) deficiency.
Findings:
- This case highlights a rare co-diagnosis of HUVS and CF1 deficiency in an adolescent.
- Genotyping confirmed the coexisting complement factor 1 deficiency.
Implications:
- The potential role of CF1 deficiency in HUVS development requires further investigation.
- This case expands the understanding of rare complement deficiencies and their association with vasculitic syndromes.
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