Hypocomplementemic urticarial vasculitis syndrome, Jaccoud's syndrome, valvulopathy: a new syndromic combination.
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.
Department of Rheumatology, Medical School Xavier-Bichat, Bichat Hospital, Paris, France.
E
E
Palazzo
P
P
Bourgeois
O
O
Meyer
M
M
De Bandt
M
M
Kazatchkine
M F
MF
Kahn
D000328:Adult; D003165:Complement System Proteins / Q000032:analysis*; D005260:Female; D006349:Heart Valve Diseases / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007592:Joint Diseases / Q000150:complications* / Q000276:immunology; D008297:Male; D008943:Mitral Valve / Q000473:pathology / Q000503:physiopathology; D013577:Syndrome; D014581:Urticaria / Q000097:blood / Q000150:complications* / Q000276:immunology; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000097:blood / Q000150:complications* / Q000276:immunology
D003165:Complement System Proteins