Mucocutaneous lymph node syndrome, or Kawasaki disease, is an acute febrile syndrome noted predominately in children less than 10 years of age.
Epidemiology. The entity, first recognized in Japanese children, demonstrates a predilection for Japanese children or American children with a Japanese background. American cases reported to the CDC indicate that the yearly incidence of Kawasaki disease is three times higher in Asian American children than in black children and more than six times higher in Asian American children than in white children, all under the age of eight, per 100,000. The incidence of the disease is higher in boys and associated with more serious complication in males.
Pathogenesis. The causative entity of the disease is unknown, although an infectious agent is suspected. There is no evidence that the disease is transmissible from person to person. Rickettsia-like bodies have been found in the tissues of some patients, although serologic tests have been negative for Rickettsia.
Clinical Manifestations. The disease is characterized by an early presentation of oropharyngeal inflammation, septic appearance with fever, and cervical adenitis. The appearance of a generalized rash with desquamation occurring over the trunk, with increased cervical adenopathy, follows the early signs. Coronary artery aneurysms and other cardiovascular complications, including coronary thrombosis, myocarditis, pericar-dial effusions, dysrhythmias, and mitral valve disease, may occur. Aneurysm development may occur in up to 20% of patients.
Diagnosis. Clinical manifestations noted above comprise the predominant criteria required for diagnosis in the absence of another known disease process. A thrombocytosis with elevated peripheral white cell count are associated laboratory findings.
Treatment. The administration of high-dose intravenous gamma globulin plus aspirin appears effective in preventing coronary artery aneurysm formation. This therapy has not been completely effective in eliminating all cardiovascular abnormalities, particularly those involving the coronary arteries.
Please see Chapter 9 for a more detailed discussion of Kawasaki disease.
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