Hypocomplementemic urticarial vasculitis, jaccoud's arthropathy, valvular heart disease, and reversible tracheal stenosis: a surfeit of syndromes.

Abstract

We describe a patient who, during 29 years of observation, manifested polyarthralgia and polyarthritis leading to progressive deformity of the joints of hands and feet (without loss of cartilage or erosion of bone); persistent urticaria made worse by cold and accompanied by hypocomplementemia; and progressive cardiac valvular disease with mitral and aortic stenosis and regurgitation. In 1996, she developed subglottic tracheal stenosis that resolved by the end of 1997 without a change in treatment, which has consisted of low dose azathioprine, glucocorticoid, and nonsteroidal antiinflammatory drugs. Tests for cryoprecipitable protein, antineutrophil cytoplasmic antibodies, antinuclear antibody, and rheumatoid factor were negative. Skin biopsy was consistent with "leukocytoclastic vasculitis." The pathogenesis of this remarkable combination of syndromes is unknown.