Kawasaki disease

Background

• A self-limited vasculitis of unknown etiology that occurs primarily in children

Diagnostic Criteria

• "Warm CREAM" mnemonic: fever plus conjunctivitis, rash, extremity involvement, adenopathy, and mucosal involvement
• Fever persisting at least 5 days, with 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‡

Incomplete Kawasaki disease

• Do not meet the full criteria above

Investigations

• CBC shows granulocyte-predominant leukocytosis with a normochromic, normocytic anemia
  • Can have thrombocytopenia
• D-dimer may be elevated
• Liver enzymes elevated in a third of patients, mild or moderate
• Hypoalbuminemia, particularly in severe acute disease
• Sterile pyuria in 80%
• Echocardiogram, mainly for coronary aneurysm
  • Can also see LV dilatation, systolic dysfunction, pericardial effusion, mitral or other valve regurgitation

Differential Diagnosis

• Viral infections: measles, adenovirus, enterovirus, Epstein-Barr virus
• Scarlet fever
• Staphylococcal scalded skin syndrome
• Toxic shock syndrome
• Bacterial cervical lymphadenitis
• Drug hypersensitivity reactions
• Stevens-Johnson syndrome
• Juvenile rheumatoid arthritis
• Rocky Mountain spotted fever
• Leptospirosis
• Mercury hypersensitivity reaction (acrodynia)

Management

• IVIG plus aspirin, reduces the rate of coronary artery aneurysm