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CASE REPORT article

Front. Pediatr.
Sec. Pediatric Immunology
Volume 12 - 2024 | doi: 10.3389/fped.2024.1448094

Hypocomplementemic Urticarial Vasculitis Syndrome in a Pediatric Patient with Complement Factor 1 Deficiency

Provisionally accepted
Sallie Lin Sallie Lin Dina Kafisheh Dina Kafisheh Melissa E. Elder Melissa E. Elder *
  • University of Florida, Gainesville, United States

The final, formatted version of the article will be published soon.

    Urticarial vasculitis (UV) is a type III hypersensitivity reaction, characterized by immune complex deposition in small vessels leading to complement activation. Hypocomplementemia urticarial vasculitis syndrome (HUVS) represents the most severe form of UV, manifesting as chronic and recurrent urticarial skin lesions with leukocytoclastic vasculitis (LCV) on histology, hypocomplementemia, anti-C1q antibodies, and systemic organ involvement. This case study focuses on an adolescent who initially presented with invasive pneumococcal infection and was later diagnosed with two rare disorders: HUVS and co-existing complement factor 1 (CF1) deficiency by genotyping. The role of CF1 deficiency in development of HUVS in this patient is uncertain but has not previously been described.

    Keywords: urticarial vasculitis., HUVS, Complement deficiency, CF1, Pneumococcal infection, C1q antibody

    Received: 12 Jun 2024; Accepted: 30 Aug 2024.

    Copyright: © 2024 Lin, Kafisheh and Elder. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Melissa E. Elder, University of Florida, Gainesville, United States

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