Kawasaki disease (KD), formerly known as mucocutaneous lymph node syndrome and infantile polyarteritis nodosa, is an acute febrile vasculitis of childhood. It causes severe vasculitis of all blood vessels but predominantly affects the medium-sized arteries, with a striking predilection for the coronary arteries.
The cause of the illness remains unknown, but epidemiologic and clinical features strongly support an infectious origin. These features include the young age group affected, epidemics with wavelike geographic spread of illness, the self-limited nature of the acute febrile illness, and the combination of clinical features of fever, rash, enanthem, conjunctival injection, and cervical lymphadenopathy.
EPIDEMIOLOGIC CASE DEFINITION (CLASSIC CLINICAL CRITERIA)
- Fever persisting at least 5 days
- Presence of at least 4 principal features:
- Changes in extremities
- Acute: Erythema of palms, soles; edema of hands, feet
- Subacute: Periungual peeling of fingers, toes in weeks 2 and 3
- Polymorphous exanthem
- Bilateral bulbar conjunctival injection without exudate
- Changes in lips and oral cavity: Erythema, lips cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosae
- Cervical lymphadenopathy (>1.5 cm diameter), usually unilateral
- Exclusion of other diseases with similar findings
OTHER CLINICAL AND LABORATORY FINDINGS
- Cardiovascular findings
- Congestive heart failure, myocarditis, pericarditis, valvular regurgitation
- Coronary artery abnormalities
- Aneurysms of medium-size noncoronary arteries
- Raynaud phenomenon
- Peripheral gangrene
- Musculoskeletal system
- Arthritis, arthralgia
- Gastrointestinal tract
- Diarrhea, vomiting, abdominal pain
- Hepatic dysfunction
- Hydrops of gallbladder
- Central nervous system
- Extreme irritability
- Aseptic meningitis
- Sensorineural hearing loss
- Genitourinary system
- Urethritis/meatitis
- Other findings
- Erythema, induration at BCG inoculation site
- Anterior uveitis (mild)
- Desquamating rash in groin
LABORATORY FINDINGS IN ACUTE KAWASAKI DISEASE
- Leukocytosis with neutrophilia and immature forms
- Elevated erythrocyte sedimentation rate (ESR)
- Elevated C-reactive protein (CRP)
- Anemia
- Abnormal plasma lipids
- Hypoalbuminemia
- Hyponatremia
- Thrombocytosis after week 1
- Sterile pyuria
- Elevated serum transaminases
- Elevated serum gamma glutamyl transpeptidase
- Pleocytosis of cerebrospinal fluid
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