Kawasaki disease--a cause of vasculitis in children

Two cases of Kawasaki disease (mucocutaneous lymph node syndrome) with peripheral vasculitis of the extremities are described. Ischemia began 2-3 weeks after the onset of symptoms and was present for greater than 24 h before hospitalization. Despite supportive therapy, gangrene of the extremities resulted in amputation. The vasculitis causes inflammation and occlusion of vessels, and therefore therapy aimed at arterial dilatation may not be beneficial. Early recognition of ischemia and treatment with correction of hypovolemia, anticoagulation, and hyperbaric therapy may be useful. Steroids may decrease peripheral vasculitis, but there is also an associated increased risk of coronary aneurysms.