What are the principal clinical features of Kawasaki disease?

Updated: Jul 29, 2018
  • Author: Tina K Sosa, MD; Chief Editor: Russell W Steele, MD  more...
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Most children with KD are brought to medical attention because of prolonged fever. There are two forms of KD: complete and incomplete. Diagnosis of complete KD requires fever of at least 5 days' duration along with 4 or 5 of the principal clinical features. The most recent version of the AHA guidelines suggest that if a patient presents with 4 or more of the principal criteria, KD can be diagnosed on day 4 of fever. [2] Experienced clinicians who have treated many KD patients may establish diagnosis before day 4, and patients who present with coronary artery disease can be diagnosed if they have at least 3 of the 5 major diagnostic criteria. [56]  The principal clinical features are as follows:

  • Extremity changes: 1) Erythema of the palms and soles, sometimes accompanied by firm and painful induration of the hands or feet that can impede ambulation 2) Desquamation of the fingers and toes which begins in the periungual region, within 2-3 weeks after the onset of fever 3) Beau's lines (deep transverse grooves across the nails) may be present 1-2 months after fever onset

  • Polymorphous rash: Typically diffuse and maculopapular, but can take on a variety of forms. Bullous, vesicular, and petechial rashes are not consistent with KD. 

  • Oropharyngeal changes: 1) Erythema, fissuring, bleeding, and/or crusting of the lips; 2) Strawberry tongue with prominent fungiform papillae; 3) Diffuse erythema of the oropharyngeal mucosa

  • Bilateral, nonexudative, limbic sparing, painless bulbar conjunctival injection (>90% of patients)

  • Acute unilateral nonpurulent cervical lymphadenopathy with lymph node diameter of at least 1.5 cm (least common criteria present)

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