Definition of Kawasaki disease

Heart Disease (Coronary Artery Disease) Slideshow

Kawasaki disease: A disease that has nothing to do with the motor bike of the same name but is a syndrome of unknown origin that mainly affects young children, causing fever, reddening of the eyes (conjunctivitis), lips and mucous membranes of the mouth, ulcerative gum disease (gingivitis), swollen glands in the neck (cervical lymphadenopathy) and a rash that is raised and bright red (maculoerythematous) in a glove-and-sock fashion over the skin of the hands and feet which becomes hard, swollen (edematous) and peels off. Also called the mucocutaneous lymph node syndrome.

The classic diagnostic criteria for Kawasaki disease are:

• Both eyes are red, usually without drainage or crusting.
• Lips and mouth are often bright red, and the top layer of the tongue peels off, leaving the tongue red and glossy.
• Hands and feet may be red and swollen. In the second week, skin under the fingernails and toenails begins to peel.
• A rash is usually present and often worse in the groin area.
• Lymph nodes in the neck may be swollen.

However, some children with Kawasaki disease do not fulfill these classic criteria and yet are at risk for developing coronary artery aneurysms, an abnormal ballooning of a coronary vessel. Children with Kawasaki disease who are not treated within the first week to 10 days of the onset of fever have five times the risk of developing coronary artery aneurysms.

The name of mucocutaneous lymph node syndrome is quite descriptive because the disease is characterized by the typical changes in the mucus membranes that line the lips and mouth and by the enlarged and tender lymph glands. The syndrome was first described in the late 1960's in Japan by the pediatrician Tomisaku Kawasaki.

Kawasaki disease affects the vascular system, and is now the main cause of acquired heart disease in children. It is most common in people of Asian descent, and is both more common and more deadly in males.

Treatment is usually with with high-dose intravenous gamma globulin, also called intravenous immunoglobulin (IVIG),