Kawasaki disease (KD), or mucocutaneous lymph node syndrome, is a multisystem vasculitis that affects infants and children. The disease occurs primarily in children younger than 6 years. It is classically characterized by fever lasting at least 5 days (usually longer) plus 4 of the 5 following 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)
Eye findings are present in over 90% of cases of KD. The most common finding is a painless bilateral nonexudative bulbar conjunctivitis with sparing of the limbus. Anterior uveitis can also be seen in the first week of fever. Less commonly, patients will have subconjunctival hemorrhages or punctate keratitis. One case report demonstrated retinal vasculitis and retinal detachment.
Most individuals affected by KD follow a benign disease course. However, coronary artery aneurysms develop in 20%-25% of untreated patients. Treatment is aimed at decreasing the risk of developing coronary artery abnormalities and preventing complications from these abnormalities.