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  1. Ian K Maconochie
  1. Correspondence to:
    Dr I K Maconochie
    Department of Paediatric Accident and Emergency, St Mary’s Hospital, London W2 1NY, UK;

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Dr Tomisaku Kawasaki published a case series of 50 children in 19671 who were febrile and all had a rash, non-exudative conjunctivitis, erythema of the palms and soles of the feet, and cervical lymphadenopathy. This constellation of signs Dr Kawasaki termed “acute febrile mucocutaneous syndrome”; however the eponym Kawasaki disease has been accepted worldwide.

We consider the difficulties in diagnosis and treatment presented by this condition and examine a recently published clinical guideline of its management.


Kawasaki disease is a systemic vasculitis predominantly affecting children under the age of 5 years. It has a number of classic clinical features required for diagnosis.

In 1990 the American Heart Association committee on rheumatic fever, endocarditis, and Kawasaki disease2 gave the case definition that has been generally accepted—ie, a febrile illness of at least five days with at least four of the five following signs and no other reasonable cause for the findings:

  • Bilateral conjunctival injection – (there is no corneal ulceration but there may be a concomitant anterior uveitis on slit lamp examination)

  • Oral changes (erythema of lips or oropharynx, strawberry tongue due to prominent papillae, or fissuring of the lips) (fig 1)

  • Peripheral extremity changes (oedema, erythema, or generalised or periungal desquamation); erythema is seen in the first week whereas desquamation begins about 14–21 days after the onset of the illness (fig 2)

  • Rash – this starts in the first few days; it is often diffuse and polymorphic and lasts a week before fading. Vesicles are rarely seen; however, the rash can appear macular, maculopapular, urticarial, scarlettina or even morbilliform (fig 3)

  • Cervical lymphadenopathy is found in about 50% of cases; most often there is a painful solitary enlarged lymph gland, > 1.5 cm in diameter

Figure 1

Mucous membrane changes (injected or fissured lips, redness of pharynx, strawberry-like tongue). Reproduced …

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  • I K Maconochie was involved as a reviewer in Oates-Whitehead R, Baumer J, Haines L et al. Intravenous immunoglobulin for the treatment of Kawasaki disease in children (Cochrane Review). In: The Cochrane Library, Issue 4, 2003. Chichester: John Wiley & Sons, Ltd; and the Cochrane review of the use of salicylate for the treatment of Kawasaki disease in children.