Kawasaki's Disease (Mucocutaneous Lymph Node Syndrome)

Kawasaki's disease is an idiopathic disease of children less than 5 years of age. It is characterized by a systemic illness and usually has a cutaneous manifestation. To make the diagnosis, fever must be present for 5 or more days.

Causes

Infectious disease caused by bacteria superantigens and virus: because Kawasaki disease is rarely seen in adults, this suggests that adults may have developed immunity to the causative agent.

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 important aspect of the disease. Kawasaki disease can cause inflammation of blood vessels in the coronary arteries, which can lead to aneurysms. Such aneurysms can lead to heart attack, even in young children.

Symptoms

• Sore throat
• Headache
• Malaise
• Nausea, vomiting, diarrhea
• Rash

Signs

• The diagnosis is made when any four of the following physical findings are found: cervical lymphadenopathy, dry, fissured lips, strawberry tongue, pharyngitis, peripheral edema or erythema, desquamation of the finger tips.
• The conjunctivae are usually injected but without exudate.
• Rash may be any nonvesicular rash, although usually is maculopapular. It commonly involves the perineum.

Treatment

Your doctor will want to begin initial treatment for Kawasaki disease as soon as possible after the appearance of signs and symptoms, preferably while your child still has a fever. The goals of initial treatment are to lower fever and inflammation and prevent heart damage.

To accomplish those goals, your child's doctor may recommend:

• Infusion of gamma globulin through a vein (intravenously) can lower the risk of coronary artery abnormalities. Intravenous gamma globulin is the standard treatment for Kawasaki disease and is administered in high doses. The child's condition usually greatly improves within 24 hours of treatment with IV gamma globulin.
• High doses of aspirin can reduce the fever, rash, joint inflammation and pain and help prevent blood clots from forming.