Kawasaki syndrome

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 4A44.5

  • Kawasaki 1967

  • Morbus Kawasaki, mucocutaneous lymph node syndrome, acute febrile mucocutaneous lymphadenopathy syndrome

  • Infection-associated diffuse vasculitis, which occurs predominantly in childhood
  • Characteristically affects the small and medium-sized vessels
    • Frequently affects the coronary arteries

  • Highest incidence is in Asia
  • Frequent vasculitis in childhood
  • 80% of patients are 1-5 years old

  • In the context of an infection, there is a cell-mediated reaction against endothelial antigens of the blood vessels (preferably medium-sized arteries).

  • 6 main symptoms
  1. High fever, without response to antibiotics
  2. Conjunctival involvement with photophobia and increased vascular injection
  3. Stomatitis (strawberry tongue
  4. Palmar and plantar erythema
  5. Polymorphic exanthema: scarlatiniform, morbilliform
  6. Cervical lymphadenopathy
  • Cardiac involvement (myocarditis/coronariitis/aneurysm)
  • Meningism
  • Bronchitis
  • Gastroenteritis

  • History
  • Clinical
  • Laboratory: elevated ESR, CRP, alpha2-globulins, leukocytosis, thrombocytosis, anaemia; positive detection of endothelial cell antibodies (AECA)
  • Biopsy
  • EGC/echocardiography, if necessary. Coronary angiography
  • To confirm the diagnosis, it requires the presence of 5 of the 6 major symptoms or 4 major symptoms and evidence of coronary aneurysm

  • Typical signs of vasculitis of the medium-sized and arteries
  • Necrotising vasculitis

  • Coronary aneurysms (20% of patients), myocardial infarction, myocarditis

  • If therapy is given, mortality is 1%

  1. Bayers S, Shulman ST, Paller AS. Kawasaki disease: part I. Diagnosis, clinical features, and pathogenesis. J Am Acad Dermatol. 2013;69; 501 e501-511; quiz 511-502. 
  2. Kawasaki T. Kawasaki disease. Int J Rheum Dis. 2014;17; 597-600. 
  3. Kawasaki T, Naoe S. History of Kawasaki disease. Clin Exp Nephrol. 2014;18; 301-304. 
  4. Seaton KK, Kharbanda A. Evidence based management of Kawasaki disease in the emergency department. Pediatr Emerg Med Pract. 2015;12; 1-20; quiz 21.