Kawasaki disease
Key points
- Kawasaki disease is a rare condition in children that involves inflammation of the blood vessels.
- Kawasaki disease occurs most frequently in Japan, where the disease was first discovered, but it occurs in all ethnicities.
- Kawasaki disease often begins with a high and persistent fever greater than 102°F, often as high as 104°F.
- There are no tests that specifically diagnose Kawasaki disease.
Alternative names
Mucocutaneous lymph node syndrome; Infantile polyarteritis
Definition
Kawasaki disease is a rare condition in children that involves inflammation of the blood vessels. The condition is usually accompanied by a fever that lasts at least 5 days. Other classic symptoms may include red eyes, lips, and mouth; rash; swollen and red hands and feet; and swollen lymph nodes.
Causes, incidence, and risk factors
Kawasaki disease occurs most frequently in Japan, where the disease was first discovered, but it occurs in all ethnicities. In the United States, where Kawasaki disease is the leading cause of acquired heart disease in children, over 4,000 children develop it each year. 80% of patients are younger than 5 years of age.
Kawasaki disease is a poorly understood illness. The cause has not been determined. It may be an autoimmune disorder precipitated by unknown outside factors. The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels, and the heart.
The heart's involvement and complications are, by far, the most important aspect of the disease. Kawasaki disease can cause inflammation of blood vessels in the coronary arteries, which can lead to aneurysms. (An aneurysm is a weakened area of blood vessel that balloons out.) Such aneurysms can lead to heart attack, even in young children (rarely).
Symptoms
Kawasaki disease often begins with a high and persistent fever greater than 102°F, often as high as 104°F. A persistent fever lasting at least five days is considered a hallmark sign. The fever may persist steadily for up to two weeks and is not very responsive to normal doses of acetaminophen or ibuprofen.
Other symptoms often include:
- Extremely bloodshot or red eyes (without pus or drainage)
- Bright red, chapped, or cracked lips
- Red mucous membranes in the mouth
- Strawberry tongue, white coating on the tongue, or prominent red bumps on the back of the tongue
- Red palms of the hands and the soles of the feet
- Swollen hands and feet
- Skin rashes, NOT blister-like, on the trunk
- Peeling skin in the genital area, hands, and feet (especially around the nails, palms, and soles)
- Swollen lymph nodes (frequently only one lymph node is swollen), particularly in the neck area.
- Joint pain and swelling, frequently symmetrical
Written and revised by
David Cohen, M.D.
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