High fever for >5 days; rash; cervical adenopathy; bulbar conjunctiva injection; fissured lips; strawberry tongue; extremity erythema, swelling, or periungual desquamation; [ID, p. 1552]
Kawasaki disease is more common in children with Japanese or Korean heritage and rare in children over 8 years of age. National epidemics in Japan occurred in 1979, 1985, and 1987. [ID, p. 1551-6]
Like scarlet fever and toxic shock syndrome, it appears to be caused by toxin-producing bacteria. Effects on the heart, including myocarditis and arterial aneurysms, are the major complications. Laboratory findings include leukocytosis, elevated sedimentation rate, and abnormal liver function tests. A swollen and inflamed gallbladder may cause obstructive jaundice. [ID, p. 1551-6] Effects on the heart (coronary artery vasculitis) begin in the subacute phase about 1-4 weeks after onset. Other findings may include abdominal pain, diarrhea, anterior uveitis, aseptic meningitis, arrhythmias, and pericarditis. [Merck Manual, p. 2754] 80% of patients are <5 years old. Diagnostic criteria include 3 days of fever and at least 4 of the following 5 conditions: 1.) bilateral conjunctivitis; 2.) changes in the oral mucosa (erythema, fissuring, strawberry tongue); 3.) changes in the extremities (erythema, edema, or desquamation); 4.) generalized rash; and, 5.) cervical adenopathy (least common). The three phases (and their durations) are acute febrile (1-2 weeks), subacute (2-4 weeks), and convalescent (1-6 weeks). Common symptoms are irritability, vomiting, cough, diarrhea, rhinorrhea, abdominal pain, and joint pain. Liver enzymes may be elevated. Almost all patients have myocarditis. Other inflammatory complications are aseptic meningitis, anterior uveitis, cranial neuropathy, arthritis, and pancreatitis. A fine pustular eruption has been reported. [PPID, p. 3527-9] About 1-2% of patients have myocardial infarction during the acute illness. [Cecil, p. 1753] Patients typically present with high fever and a polymorphic eruption that is scarlatiniform, urticarial, morbilliform, or targetoid. [Cecil, p. 2622] Laboratory abnormalities include leukocytosis acutely, mild normocytic anemia, thrombocytosis, sterile pyuria (if nephritis), elevated liver enzymes (if hepatitis), and CSF pleocytosis (if meningitis). [Merck Manual, p. 2754-5]
Clinical; Common laboratory abnormalities are elevated sedimentation rate, C-reactive proteins, and platelet count; [CCDM, p. 327]
REFERENCES FOR CASES/YEAR
1. (US) See global explanation.
2. (Global) Rate in children under 5 is >200 per 100,000 in Japan and 20 per 100,000 in the USA; Other rates are 113 (South Korea), 69 (Taiwan), and 55 (Beijing, China); [CCDM, p. 327] If assume that 1/30 of population is <5 years, then rates per year are 20 X 3000 = 60,000 and 60,000/30 = 2000 for US and 200 X 1260 = 252,000 and 252,000/30 = 8,400 for Japan; Guesstimate global cases/year as 20 X US rate = 40,000;