Last Updated: 2023-07-07
Author(s): Anzengruber F., Navarini A.
- 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
- High fever, without response to antibiotics
- Conjunctival involvement with photophobia and increased vascular injection
- Stomatitis (strawberry tongue
- Palmar and plantar erythema
- Polymorphic exanthema: scarlatiniform, morbilliform
- Cervical lymphadenopathy
- Cardiac involvement (myocarditis/coronariitis/aneurysm)
- Laboratory: elevated ESR, CRP, alpha2-globulins, leukocytosis, thrombocytosis, anaemia; positive detection of endothelial cell antibodies (AECA)
- 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%
- 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.
- Kawasaki T. Kawasaki disease. Int J Rheum Dis. 2014;17; 597-600.
- Kawasaki T, Naoe S. History of Kawasaki disease. Clin Exp Nephrol. 2014;18; 301-304.
- Seaton KK, Kharbanda A. Evidence based management of Kawasaki disease in the emergency department. Pediatr Emerg Med Pract. 2015;12; 1-20; quiz 21.