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The cause of Kawasaki disease has not been determined. The incidence is high in Japan where the disease was first described and is recognized now more frequently in the United States. Other risk factors than age are unknown. Kawasaki disease is predominatly a disease of young children, with 80% of patients younger than 5 years of age.

Kawasaki disease is a poorly understood illness. It appears in many respects to be an immune vasculitis (an autoimmune disorder). It is precipitated by unknown outside factors. The disorder affects the mucus membranes, lymph nodes, lining of the blood vessels and the heart. The cardiac involvement and complications are, by far, the most important aspect of the disease. Kawasaki disease can cause vasculitis (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. These aneurysms can lead to myocardial infarction (heart attack) even in young children (rarely). About 20 - 40% of children with Kawasaki disease will have evidence of vasculitis with cardiac involvement.

Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal doses of acetaminophen or ibuprofen. The fever may persist steadily for up to two weeks. The children develop red eyes, red mucous membranes in the mouth, red cracked (fissured) lips, a "strawberry tongue", and swollen lymph nodes. Skin rashes may occur early in the disease and peeling of the skin in the groin (genital area), hands, and feet (especially around the nails and on the palms and soles) may occur.

The changes in the coronary arteries can only be demonstrated by testing. Echocardiography (non-invasive) or angiography, a study in which dye is injected into the blood stream and the heart and its coronary arteries viewed or X-ray may be used.