[Hypocomplementemic urticarial vasculitis syndrome].
METHODS
A 40-year-old woman was admitted with recurrent membranoproliferative glomerulonephritis. For many years she had been suffering from joint pains and attacks of angio-oedema of the upper and lower lip. Physical examination was unremarkable except for marked oedema and urticaria of the trunk and limbs.


METHODS
Skin biopsy revealed urticaria with vasculitis. Complement fractions C3, C4 and C1q were reduced, and C1q antibodies were demonstrated.


METHODS
The findings of urticaria with vasculitis and reduced complement levels as cardinal symptoms together with joint pains, glomerulonephritis and C1q antibodies as minor symptoms were diagnostic of hypocomplementaemic vasculitic urticaria syndrome. The urticaria disappeared immediately when immunosuppressive treatment with prednisolone and cyclophosphamide was started, and the nephritic signs regressed.


CONCLUSIONS
Differentiation of a vasculitic and a non-vasculitic form of chronic urticaria provides an initial step in delineating the underlying disease. Severe systemic disease such as collagen disease or vasculitis is not rare in vasculitic urticaria and requires long-term immunosuppression.
Sessler
R
R
Zentrum für Innere Medizin, Abteilung für Nieren- und Hochdruckkrankheiten, Katharinenhospital, Stuttgart.
Hasche
G
G
Olbricht
C J
CJ
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D015922:Complement C1q / Q000172:deficiency / Q000276:immunology; D003165:Complement System Proteins / Q000172:deficiency*; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D013577:Syndrome; D014581:Urticaria / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D015922:Complement C1q; D003520:Cyclophosphamide; D003165:Complement System Proteins; D011239:Prednisolone