A five year-old boy has had seven days of fever up to 104F. Over the last two days, he has developed a large right-sided cervical lymph node, a maculopapular rash, and injected sclera. His lips are red and cracked.

What are the clinical criteria for Kawasaki Disease (KD)?

Diagnosis of Kawasaki Disease requires fever for more than five days plus at least four of the following:

• Bilateral conjunctival injection (occurs in 80-90% of patients; typically limbic-sparing)
• Oropharyngeal mucous membrane changes (80-90%; injected and / or fissured lips, strawberry tongue, injected pharynx)
• Peripheral extremity changes (80%; Erythema and / or edema of the hands and feet or periungual desquamation)
• Polymorphous rash (>90%)
• Cervical lymphadenopathy with node >1.5 cm (50%)

What is the major complication of Kawasaki Disease and how is it prevented?

In 25% of untreated patients, coronary artery aneurysms develop. KD is the leading cause of childhood acquired heart disease worldwide. Aneurysms may lead to coronary insufficiency, thrombus, myocardial infarction, and death. Treatment with IVIG (usually during the acute, febrile phase) reduces the incidence of aneurysms by 85%. It also reduces the incidence of giant aneurysms and overall mortality.