Kawasaki syndrome

Kawasaki syndrome is an acute mucocutaneous syndrome of young children characterized by prolonged fever, rash, mucositis, cervical adenitis, and changes of the peripheral extremities. Unlike most other illnesses, the rash is not distinctive and is remarkable for its variable morphology, site of onset, and pattern of progression. The cause and pathogenesis of Kawasaki syndrome remain a mystery, but most investigators favor a microbial agent as the trigger, followed by an immune-mediated vasculitis that has a predilection for the coronary arteries. Serious complications include coronary artery aneurysms, aneurysmal thrombosis, and death. Aspirin has been the most widely used treatment for Kawasaki syndrome, but it has not been shown to alter the basic pathology. Treatment with aspirin plus intravenous gamma globulin can reduce the frequency of coronary artery abnormalities. Definitive therapy of Kawasaki syndrome awaits the discovery of its cause and pathogenesis.