Kawasaki syndrome is a potentially fatal inflammatory disease that affects
several organ systems in the body, including the heart, circulatory
system, mucous membranes, skin, and immune system. As of 2004 its cause
In the 1960s, Tomisaku Kawasaki noted a characteristic cluster of symptoms
in Japanese schoolchildren. Ultimately named for Kawasaki, the disorder
was subsequently found worldwide. Kawasaki syndrome, also called
mucocutaneous lymph node syndrome (MLNS), is an inflammatory disorder with
potentially fatal complications affecting the heart and its larger
Kawasaki syndrome occurs primarily in infants and children; about 80
percent of diagnosed patients are under the age of five. On rare
occasions, the disorder has been diagnosed in teenagers or adults. Nearly
twice as many males are affected as females. Although persons of Asian
descent are affected more frequently than either black or white
individuals, there does not appear to be a distinctive geographic pattern
of occurrence. Although the disease usually appears in individuals, it
sometimes affects several members of the same
and occasionally occurs in small epidemics. About 3,000 cases are
diagnosed annually in the United States.
Causes and symptoms
The specific cause of Kawasaki syndrome was as of 2004 unknown, although
the disease resembles an infectious illness in many ways. It has been
suggested that Kawasaki syndrome represents an allergic reaction or other
unusual response to certain types of infections. Some researchers think
that the syndrome may be caused by the interaction of an immune cell,
called the T cell, with certain poisons (toxins) secreted by bacteria.
Kawasaki syndrome has an abrupt onset, with
as high as 104°F (40°C) and a rash that spreads over the
patient's chest and genital area. The fever is followed by a
characteristic peeling of the skin beginning at the fingertips and
toenails. In addition to the body rash, the patient's lips become
very red, with the tongue developing a "strawberry"
appearance. The palms, soles, and mucous membranes that line the eyelids
and cover the exposed portion of the eyeball (conjuntivae) become
purplish-red and swollen. The lymph nodes in the patient's neck may
also become swollen. These symptoms may last from two weeks to three
months, with relapses in some patients.
In addition to the major symptoms, about 30 percent of patients develop
or arthritis, usually in the large joints of the body. Others develop
, dry or cracked lips,
, or an inflammation of the membranes covering the brain and spinal cord (
). A few patients develop symptoms of inflammation in the liver
(hepatitis), gallbladder, lungs, or tonsils.
About 20 percent of patients with Kawasaki syndrome develop complications
of the cardiovascular system. These complications include inflammation of
the heart tissue (myocarditis), disturbances in heartbeat rhythm
(arrhythmias), and areas of blood vessel dilation (aneurysms) in the
coronary arteries. Other patients may develop inflammation of an artery
(arteritis) in their arms or legs. Complications of the heart or arteries
begin to develop around the tenth day after the illness begins, when the
fever and rash begin to subside. A few patients may develop gangrene (the
death of soft tissue) in their hands and feet. The specific causes of
these complications were as of 2004 not known.
Because Kawasaki syndrome is primarily a disease of infants and young
children, the disease is most likely to be diagnosed by a pediatrician.
The physician will first consider the possible involvement of other
diseases that cause fever and skin
Rocky Mountain spotted fever
(a disease carried by cats), juvenile rheumatoid arthritis, and a
blistering and inflammation of the skin caused by reactions to certain
medications (Stevens-Johnson syndrome).
Once other diseases have been ruled out, the patient's symptoms
will be compared with a set of diagnostic criteria. The patient must have
a fever lasting five days or longer that does not respond to
, together with four of the following five symptoms:
Given the unknown cause of this syndrome, there are no laboratory tests
that can confirm the diagnosis. The following test results, however, are
associated with the disease:
In addition to these tests, it is important to take a series of
echocardiograms during the course of the illness because 20 percent of
Kawasaki patients develop coronary aneurysms or arteritis that will not
appear during the first examination.
An angiogram showing abnormal coronary arteries in a child suffering
from Kawasaki's disease. The coronary arteries bulge into
balloon shapes, called aneurysms, along their lengths.
(Photograph by Mehau Kulyk. Photo Researchers, Inc.)
Kawasaki syndrome is usually treated with a combination of aspirin, to
control the patient's fever and skin inflammation, and high doses
of intravenous immune globulin to reduce the possibility of coronary
artery complications. Some patients with heart complications may be
treated with drugs that reduce blood clotting or may receive corrective
Follow-up care includes two to three months of monitoring with chest
, electrocardiography, and echocardiography. Treatment with aspirin is
often continued for several months.
Most patients with Kawasaki syndrome will recover completely, but about
1–2 percent die as a result of blood clots forming in the coronary
arteries or as a result of a heart attack. Deaths are sudden and
unpredictable. Almost 95 percent of fatalities occur within six months of
infection, but some have been reported as long as 10 years afterward.
Long-term follow-up of patients with aneurysms indicates that about half
show some healing of the aneurysm. The remaining half has a high risk of
heart complications in later life.
—A weakened area in the wall of a blood vessel which causes an
outpouching or bulge. Aneurysms may be fatal if these weak areas burst,
resulting in uncontrollable bleeding.
—Any deviation from a normal heart beat.
—Inflammation of an artery.
—An enlarged heart.
—Plural, conjunctivae. The mucous membrane that covers the white
part of the eyes (sclera) and lines the eyelids.
—A skin eruption associated with a disease, usually one
accompanied by fever as in Kawasaki syndrome.
—Decay or death of body tissue because the blood supply is cut
off. Tissues that have died in this way must be surgically removed.
—An inflammation of the liver, with accompanying liver cell
damage or cell death, caused most frequently by viral infection, but
also by certain drugs, chemicals, or poisons. May be either acute (of
limited duration) or chronic (continuing). Symptoms include jaundice,
nausea, vomiting, loss of appetite, tenderness in the right upper
abdomen, aching muscles, and joint pain. In severe cases, liver failure
—An infection or inflammation of the membranes that cover the
brain and spinal cord. It is usually caused by bacteria or a virus.
Mucocutaneous lymph node syndrome (MLNS)
—Another name for Kawasaki syndrome. The name comes from the key
symptoms of the disease, which involve the mucous membranes of the mouth
and throat, the skin, and the lymph nodes. MLNS is a potentially fatal
inflammatory disease of unknown cause.
—Inflammation of the heart muscle (myocardium).
—A severe inflammatory skin eruption that occurs as a result of
an allergic reaction or respiratory infection.
—A type of white blood cell that is produced in the bone marrow
and matured in the thymus gland. It helps to regulate the immune
system's response to infections or malignancy.
Rowland, Anne. "Bacteria: Diseases of Possible Infectious or
Unknown Etiology." In
Principles and Practice of Pediatric Infectious Diseases
, 2nd ed. Edited by Sarah S. Long et al. St. Louis, MO: Elsevier, 2003.
Rowley, Anne H., and Stanford T. Shulman. "Kawasaki
Nelson Textbook of Pediatrics.
Edited by Richard E. Behrman et al. Philadelphia: Saunders, 2004.
"Kawasaki Syndrome (mucocutaneous lymph node syndrome)."
South Dakota Department of Health
, November 18, 2004. Available online at
http://sss.state.sd.us/doh/Pubs/Kawasaki.htm (accessed December