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Hypocomplementemic urticarial vasculitis syndrome, Jaccoud's syndrome, valvulopathy: a new syndromic combination
E Palazzo1, P Bourgeois, O Meyer
1Department of Rheumatology, Medical School Xavier-Bichat, Bichat Hospital, Paris, France.
Insights
This study details three cases of hypocomplementemic urticarial vasculitis syndrome (HUVS) presenting with Jaccoud
Area of Science:
- Rheumatology
- Immunology
- Cardiology
Background:
- Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder.
- HUVS is characterized by cutaneous vasculitis, hypocomplementemia, and often systemic involvement.
- Jaccoud's hands deformity and cardiac valve disease are not typically associated with HUVS.
Abstract:
We describe 3 cases of hypocomplementemic urticarial vasculitis syndrome (HUVS) with Jaccoud's hands deformity and cardiac valve disease (aortic regurgitation, mitral regurgitation, mitral disease). In one case, the valve lesions required valve replacement and later a heart transplant. Valve disease and articular deformities developed 2 and 4 years, respectively, after the onset of HUVS. This as yet undescribed combination of diseases suggests a new syndrome. Pathogenesis of periarticular and cardiac lesions is unknown. The role of Clq and anti-Clq antibody is discussed.