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[Chronic urticaria and acquired complement deficiency due to a nephritic factor (C3NeF)].
L Borradori1, M Rybojad, L Weiss
1Clinique dermatologique, Centre hospitalier universitaire Saint Louis, Paris.
Schweizerische Medizinische Wochenschrift
|August 25, 1990
Summary
Chronic urticaria may involve complement system activation. A patient with chronic urticaria and a C3 nephritic factor (C3NeF) showed alternative pathway activation, suggesting a potential role for C3NeF in urticaria pathogenesis.
Area of Science:
- Immunology
- Dermatology
- Complement System
Background:
- Urticaria pathogenesis is often linked to complement activation.
- The complement system plays a crucial role in immune responses and inflammation.
Observation:
- A case study of a 47-year-old patient with chronic urticaria.
- The patient exhibited persistent low hemolytic complement activity, depressed C3 and Factor B levels.
- Alternative complement pathway activation was indicated by specific complement component levels.
Findings:
- Detection of a circulating low-affinity C3 nephritic factor (C3NeF).
- Urticarial lesions evolved from pruritic to fixed and burning, accompanied by systemic symptoms like fever and arthralgia.
- Skin biopsies revealed leukocytoclastic vasculitis, with poor response to conventional treatments but partial improvement with corticosteroids.
Implications:
- This is the first reported association of chronic urticarial vasculitis with C3NeF in the absence of glomerulonephritis or lipodystrophy.
- The findings suggest a potential novel pathogenic role for C3NeF in specific forms of chronic urticaria.
- Further research is warranted to explore the link between C3NeF and urticaria for potential therapeutic targets.