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Kawasaki disease (KD), or mucocutaneous lymph node syndrome

Kawasaki disease (KD), or mucocutaneous lymph node syndrome, is an idiopathic, smallvessel vasculitis characterized by fever and mucocutaneous inflammation. It has a peak incidence in infants aged 911 months and is extremely rare in infants younger than 3 months of age. Although usually selflimited, potentially lifethreatening coronary artery aneurysms may develop in 20%25% of children without treatment (versus less than 5% with appropriate therapy). Mortality most often occurs within the first weeks to a year after KD due to ischemic heart disease caused by myointimal proliferation within persistent aneurysms. Infants younger than 6 months of age may be at increased risk for aneurysms.

The classic case definition of KD is:

Fever lasting at least 5 days
Plus the presence of at least 4 of the following principal clinical criteria:
Bilateral bulbar conjunctival injection without exudate
Oral mucosa changes: cracked lips, "strawberry tongue," or diffuse erythema of the mucosae
Changes in the extremities: erythema, induration, or periungual peeling
Exanthem
Cervical lymphadenopathy (greater than 1.5 cm diameter)

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