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Skin lesions, angioedema, eosinophilia, and hypocomplementemia
The Journal of Pediatrics
|November 1, 1976
Summary
A child with urticaria, angioedema, and vasculitis showed eosinophilia and complement activation. Corticosteroid therapy led to remission, suggesting a link to similar adult syndromes.
Area of Science:
- Immunology
- Pediatric Rheumatology
- Dermatology
Background:
- Recurrent urticaria and angioedema can indicate underlying systemic disease.
- Vasculitis and renal disease require thorough investigation for immune system involvement.
Observation:
- A pediatric case presented with recurrent urticaria, angioedema, vasculitis, and probable renal disease.
- Marked blood eosinophilia, elevated serum IgE, circulating Clq precipitins, and hypocomplementemia were noted.
- Skin and muscle biopsies showed eosinophilic infiltration of vessel walls.
Findings:
- Complement activation via the classic pathway was evidenced by hypocomplementemia.
- Immunofluorescence revealed IgM, IgE, and C3 deposition in vessel walls.
- Disease exacerbations correlated with increased eosinophils and decreased C4/C3 levels.
Implications:
- The patient's presentation shares features with adult syndromes of skin lesions, angioedema, and hypocomplementemia.
- Corticosteroid therapy induced remission, highlighting its therapeutic potential.
- This case underscores the importance of considering immune-mediated vasculitis in pediatric patients with complex presentations.