Kawasaki disease is an acute, necrotizing vasculitis of unknown etiology. The condition primarily affects children under the age of five and is more common among those of Asian descent. The disease is characterized by a high fever, desquamative rash, conjunctivitis, mucositis (e.g., “strawberry tongue”), cervical lymphadenopathy, as well as erythema and edema of the distal extremities. However, coronary artery aneurysms are the most concerning possible manifestation as they can lead to myocardial infarction or arrhythmias. Kawasaki disease is a clinical diagnosis, further supported by findings such as elevated ESR or evidence of cardiac involvement on echocardiography. Treatment with IV immunoglobulins and high-dose aspirin is essential and should be initiated immediately after diagnosis.
- Sex: ♂ > ♀ (1.5:1) 
- Age: : primarily children < 5 years (most common cause of acquired coronary artery disease in children) 
- Peak incidence: occurs mostly in late winter and spring
- Approx. 20 per 100,000 children
- Highest rate in children of Asian and Pacific-Islander descent
- Mortality: < 0.5% 
Epidemiological data refers to the US, unless otherwise specified.
- The exact cause of Kawasaki disease remains uncertain.
- However, it is associated with infectious and genetic factors (the prevalence is higher in patients of Asian descent and in siblings of affected children). 
Clinical diagnosis requires fever for at least 5 days and one of the following: 
- ≥ 4 other specific symptoms
- < 4 specific symptoms and involvement of the coronary arteries
- Specific symptoms include:
- Erythema and edema of hands and feet, including the palms and soles (the first week)
- Possible desquamation of fingertips and toes after 2–3 weeks
- Polymorphous rash, originating on the trunk
- Painless bilateral “injected” conjunctivitis without exudate
- Oropharyngeal mucositis
- Cervical lymphadenopathy (mostly unilateral)
- Nonspecific symptoms may precede the onset of Kawasaki disease (e.g., diarrhea, fatigue, abdominal pain, arthritis, hoarseness, breathing difficulties) 
Kawasaki disease is a clinical diagnosis (see “Clinical features” above) which is supported by the following diagnostic findings:
- IV immunoglobulin (IVIG)
- High-dose oral aspirin 
- IV glucocorticoids: may be considered in addition to standard treatment, esp. in cases of treatment-refractory disease, as they lower the risk of coronary involvement
- Coronary artery aneurysm ; 
- Myocardial infarction
- Most commonly occurs within the first 2 weeks of onset of Kawasaki disease 
- Often seen in infants with untreated Kawasaki disease 
- Most commonly associated with lymphocytic myocarditis 
- Manifests with symptoms (e.g., tachypnea or diaphoresis with feeding, exertional dyspnea; ) and physical examination findings of congestive heart failure (e.g., hepatomegaly, S3 gallop, rales or crackles on lung auscultation) 
- Increased risk of sudden death 
We list the most important complications. The selection is not exhaustive.