Kawasaki disease (mucocutaneous lymph node syndrome) is a children's illness characterized by fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips and throat. These immediate effects of Kawasaki disease are rarely serious; however, long-term heart complications result in some cases and can be seen as early as two weeks after onset of the disease.

Named after Dr. Tomisaku Kawasaki, a Japanese pediatrician, the disease has probably been in existence for a long time, but was not recognized as a separate entity until 1967. The incidence is higher in Japan than in any other country. In the United States it is more frequent among children of Asian-American background, but can occur in any racial or ethnic group. The disease is relatively common, and in the United States it is a major cause of heart disease in children. In recent years, it has tended to occur in localized outbreaks, most often in the late winter or spring, but is seen year-round. Kawasaki disease almost always affects children; most patients are under 5 years old, and the average age is about 2. Boys develop the illness almost twice as often as girls.

The heart may be affected in as many as one of five children who develop Kawasaki disease. Damage sometimes occurs to the blood vessels that supply the heart muscle (the coronary arteries) and to the heart muscle itself. A weakening of a coronary artery can result in an enlargement or swelling of the blood vessel wall (an aneurysm). Infants less than 1 year old are usually the most seriously ill and are at greatest risk for heart involvement. The acute phase of Kawasaki disease commonly lasts 10 to 14 days or more. Most children recover fully. The likelihood of developing coronary artery disease later in life is not known, and remains the subject of medical investigation.

The symptoms of Kawasaki disease include...

• fever
• rash
• swollen hands and feet
• irritation and redness of the whites of the eyes
• swollen lymph glands in the neck
• irritation and inflammation of the mouth, lips and throat

Doctors don't know what causes Kawasaki disease, but it doesn't seem to be hereditary or contagious. Scientists who've studied it think the evidence strongly suggests it's caused by an infectious agent such as a virus. It's very rare for more than one child in a family to develop Kawasaki disease. Less than 2 percent of children have another attack of Kawasaki disease.

In as many as 15 to 25 percent of the children with Kawasaki disease, the heart is affected. The coronary arteries or the heart muscle itself can be damaged.

Signs For Kawasaki Disease

• The lips typically become erythematous or fissured, along with inflamed oral mucosa and the presence of a 'strawberry tongue' – so-called because it is extremely erythematous with prominent papillae.
• The rash is described as a polymorphic exanthem and comes on within 3–5 days of the onset of fever. It usually begins with non-specific erythema of the soles, palms, and perineum, spreading to involve the trunk and the rest of the extremities. It is often itchy and variable in appearance, but is never vesiculo-bullous. It is usually markedly red and may appear macular, morbilliform, papular, scarlatiniform, urticarial, akin to erythema multiforme or be made up of very many tiny micropustules.
• Desquamation may affect the perineal area, moving to the fingers and then the toes. In the extremities it usually begins in the periungual region.
• The hands and feet often become red and swollen and tender before desquamation begins.
• The conjunctivitis is never associated with exudate, is bilateral and tends to spare the perilimbal area.
• Cervical lymphadenopathy is usually unilateral, non-tender and affects the anterior cervical chain.
• Cardiovascular signs are usually non-specific. Tachycardia, a hyperdynamic precordium, a gallop rhythm or a flow murmur may be present, but these signs are not unusual in febrile patients without Kawasaki disease. There are occasionally signs of valvular incompetence.
• Other possible signs include:
  * Neck stiffness due to aseptic meningitis
  * Hepatomegaly and jaundice