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Self-limited vasculitic syndrome of unknown etiology characterized by fever, cervical adenopathies, and cardiac involvement.

Kawasaki disease

Strawberry tongue in a child with Kawasaki disease.

Kawasaki disease

Palmar erytherma and desquamation of the skin in a child with Kawasaki disease.

Mucocutaneous Lymph Node Syndrome.

Asian people are at greater risk: 5000-6000 new cases per year occur in Japan versus 3000 in the United States. Males are affected 1.5 times more often than females. Kawasaki disease (KD) is currently the leading cause of acquired heart disease in the United States among children younger than 5 years. Mortality from coronary artery abnormalities, including coronary aneurysms, is reported in 20 to 25% of untreated individuals.

Etiology of KD is unknown. Several possibilities are considered, including an infectious etiology (retrovirus?), an immunologic abnormality, and even a toxic cause (carpet shampoo). Clinical and epidemiologic data support an infectious etiology, but many authors think that an autoimmune component or genetic predisposition also exists.

Necrotizing vasculitis of medium-size muscular arteries, including coronary and cerebral vessels. Arteries show focal segmental destruction, with subsequent ectasia and formation of aneurysms in 15 to 25% of children. Several infectious causes of KD have been theorized and include Ebstein-Barr virus, retroviruses, Streptococcus pyogenes and viridans, Staphylococcus, Chlamydia, Propionibacterium, and Pseudomonas species. However, conventional bacterial and viral cultures and serologic studies have not confirmed an infectious cause. Other postulated etiologic agents are immunizations, medications, and environmental agents, such as exposure to rug shampooing agents or house dust mites. The finding of a skewed T-cell receptor response in the myocardium and the coronary arteries led to a search for superantigens, such as toxic shock syndrome toxin-1 produced by Staphylococcus aureus. Immunohistochemical findings lend support to the hypothesis of a proinflammatory cell-mediated immune reaction possibly triggered by a superantigen or a conventional antigen.

Clinical diagnosis. No diagnostic laboratory tests are available. The Centers for Disease Control and Prevention in Atlanta (USA) defines a case of KD as illness in a patient with fever of 5 or more days' duration (or fever until the of administration of intravenous immunoglobulin, if this is given before the fifth day of fever), and the presence of at least 4 of the following 5 clinical signs:

  • Rash
  • Cervical lymphadenopathy (at least one lymph node ≥ 1.5 cm in diameter)
  • Bilateral conjunctival injection
  • Oral mucosal changes (injected or fissured lips, injected pharynx, “strawberry” tongue)
  • Peripheral extremity changes (erythema of palms or soles, edema of the hands or feet, or generalized or periungual desquamation).

Patients whose illness does not meet the above KD case definition but who have fever and coronary artery abnormalities are classified as ...

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