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Kawasaki disease (KD) is the leading cause of acquired heart disease is the western world and the second leading cause of vasculitis in childhood. The history of this relatively young condition has, however, seen our understanding of clinical features, aetiology and management evolve over the last six decades.
Evolution of mucocutaneous lymph node syndrome
KD was first described as a medical condition by Tomisaku Kawasaki in 1967, in a paper where he described 50 patients with systemic features and a prolonged fever, presenting over 6 years.1 He first described a group of patients from 1962 with fever and skin peeling and, over the next 2 years, he noted a consistent involvement of the eyes, skin and mucus membranes and classified these patients under the umbrella term of ‘ocular-mucocutaneous syndrome’. As case numbers grew, he hypothesised the clinical features represented a new, yet to be diagnosed pathological process which he defined as a ‘acute febrile mucocutaneous lymph node syndrome’ (MCLNS) in his 1967 paper.
In parallel, another Japanese physician, Dr Takajiro Yamamoto, began collecting cases with similar features. It was in 1968 that Yamamoto et al first labelled this condition as KD and described the triad of prolonged fever, characteristic clinical features and cardiac involvement as hallmarks of the disease in a series of 23 patients.2 The clinical features in conjunction with prolonged (>5 days) fever which determine diagnosis have remained unchanged since the publication of these seminal papers and consist of bilateral, non-suppurative conjunctivitis; cervical lymphadenopathy >1.5 cm; polymorphous rash; oral changes, including cracked lips and strawberry tongue; and peripheral changes in the hand …
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Contributors RK and PS contributed equally to the writing and editing of the manuscript.
Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Commissioned; externally peer reviewed.