Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)

An acute multisystemic vasculitis of unknown aetiology (?infective) in children usually <5 yrs presenting with an acute febrile illness. Typically targets coronary arteries.

Diagnostic features and criteria

Fever persisting for more than 5 d plus at least 4 of:

• bilateral conjunctival congestion (non-purulent)

• mucous membrane changes—dryness, redness and cracking of the lips ± erythema of tongue, buccal mucosa

• maculopapular polymorphic rash, esp. trunk, genitalia

• cervical lymphadenopathy >1.5 cm usu. unilaterial

• erythema and swelling of palms and soles

Followed by desquamation of fingertips (a characteristic).

Diagnosis with 5/6 features or 4/6 plus evidence of coronary aneurysm (plus exclusion of other diseases). It is a clinical diagnosis.

Above features may be variable/incomplete and not all present concurrently.

No specific test but elevated ESR, neutrophilia, thrombocytosis and various +ve antibody tests (e.g. antiendothelial cell). Generally benign and self-limiting but early diagnosis is important to prevent life-threatening complications, esp. coronary aneurysms (15–30% in untreated) and also myocardial infarction, pericarditis and myocarditis.

Management

• Echocardiography and ECG are indicated

• Treat with aspirin (start ASAP) and high-dose IV normal gammaglobulin

• Corticosteroid use is controversial; check with consultant if necessary

• Most children recover and the overall mortality is <3%; often benign and self-limiting