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[Hypocomplementemic urticarial vasculitis syndrome].

R Sessler1, G Hasche, C J Olbricht

  • 1Zentrum für Innere Medizin, Abteilung für Nieren- und Hochdruckkrankheiten, Katharinenhospital, Stuttgart.

Deutsche Medizinische Wochenschrift (1946)
|September 27, 2000
PubMed
Summary
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Hypocomplementemic vasculitic urticaria syndrome (HVUS) is a rare condition characterized by vasculitis and urticaria. Prompt diagnosis and immunosuppressive treatment with prednisolone and cyclophosphamide can effectively manage symptoms and prevent severe systemic complications.

Area of Science:

  • Rheumatology
  • Nephrology
  • Dermatology

Background:

  • Hypocomplementemic vasculitic urticaria syndrome (HVUS) is a rare autoimmune disorder.
  • It is characterized by recurrent urticaria, angioedema, joint pain, and glomerulonephritis.

Observation:

  • A 40-year-old woman presented with a history of joint pain and angioedema, along with marked edema and urticaria.
  • Skin biopsy confirmed urticaria with vasculitis, and laboratory tests revealed reduced complement levels (C3, C4, C1q) and positive C1q antibodies.

Findings:

  • The patient was diagnosed with hypocomplementemic vasculitic urticaria syndrome based on cardinal symptoms of urticaria with vasculitis and hypocomplementemia, along with joint pain, glomerulonephritis, and C1q antibodies.
  • Treatment with prednisolone and cyclophosphamide led to immediate resolution of urticaria and regression of nephritic signs.

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Implications:

  • Distinguishing vasculitic from non-vasculitic chronic urticaria is crucial for identifying underlying systemic diseases.
  • HVUS can be associated with severe systemic conditions like collagen diseases or vasculitis, necessitating long-term immunosuppressive therapy.