Hypocomplementemic urticarial vasculitis, Jaccoud's arthropathy, valvular heart disease, and reversible tracheal stenosis: A surfeit of syndromes

We describe a patient who, during 29 years of observation, manifested polya rthralgia and polyarthritis leading to progressive deformity of the joints of hands and feet (without loss of cartilage or erosion of bone): persisten t urticaria made worse by cold and accompanied by hypocomplementemia; and p rogressive cardiac valvular disease with mitral and aortic stenosis and reg urgitation. In 1996, she developed subglottic tracheal stenosis that resolv ed by the end of 1997 without a change in treatment, which has consisted of low dose azathioprine, glucocorticoid, and nonsteroidal antiinflammatory d rugs. Tests for cryoprecipitable protein, antineutrophil cytoplasmic antibo dies, antinuclear antibody, and rheumatoid factor were negative. Skin biops y was consistent with "leukocytoclastic vasculitis." The pathogenesis of th is remarkable combination of syndromes is unknown.