Kawasaki disease overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Kawasaki disease, also known as lymph node syndrome, mucocutaneous node disease, infantile polyarteritis and Kawasaki syndrome, is a poorly understood self-limited vasculitis that affects many organs, including the skin and mucous membranes, lymph nodes, blood vessel walls, and the heart. It does not seem to be contagious. It was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than 5 years of age. Additional risk factors in the United States include Asian race and male sex. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. Common symptoms of kawasaki disease include high grade fever, red eyes, bright red and cracked lips, red mucous membranes in the mouth, strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue, red palms of the hands and the soles of the feet, swollen hands and feet, and rash. Intravenous Immunoglobulin (IVIG) and aspirin are indicated in kawasaki disease.
Historical perspective
Kawasaki disease was first discovered by Dr. Tomisaku Kawasaki when he saw his first case of Kawasaki disease in Japan, in 1961. Later in 1967, Kawasaki published his first report of Kawasaki disease in Japanese. Dr Kawasaki also developed "Japan Kawasaki Disease Research Center" in 1990.
Classification
Patients whose illness does not meet the diagnostic criteria of Kawasaki disease case definition, but who have fever and coronary artery abnormalities are classified as atypical or incomplete Kawasaki disease. The patients of atypical or incomplete kawasaki disease, an evidence of coronary abnormalities or CAA’s must be shown on the echocardiogram.
Pathophysiology
Causes
The exact cause of kawasaki disease has not been identified. The current etiological theories center primarily on immunological causes for the disease, much research is being performed to discover a definitive toxin or antigenic substance, possibly a superantigen, that is the specific cause of the disease. There are several hypothesis for the causes of Kawasaki disease, the Infectious agents which are thought to induce kawasaki disease are, parvovirus B19, meningococcal septicemia, adenovirus, bacterial toxin–mediated, superantigens, cytomegalovirus, epstein-Barr virus, human lymphotropic virus infection, klebsiella pneumoniae bacteremia, mycoplasma pneumoniae, mite-associated bacteria, measles, propionibacterium acnes, parainfluenza type 3 virus, rotavirus infection, rickettsia species and tick-borne diseases.
Differentiating Kawasaki disease from other diseases
Different rash-like conditions can be confused with Kawasaki disease and are thus included in its differential diagnosis. The various conditions that should be differentiated from Kawasaki disease include Impetigo ,Insect bites, Measles, Monkeypox, Rubella, Atypical measles, Coxsackievirus, Acne, Syphilis, Molluscum contagiosum, Mononucleosis, Toxic erythema, Rat-bite fever, Parvovirus B19, Cytomegalovirus, Scarlet fever, Rocky Mountain spotted fever, Stevens-Johnson syndrome, Varicella-zoster virus, Chickenpox, Meningococcemia, Rickettsial pox, Meningitis, Toxic shock syndrome, Roseola Infantum (exanthem subitum), Erythema Infectiosum (Fifth Disease), Enterovirus, Dengue Fever, Drug induced rash, Infectious Mononucleosis, Pharyngoconjunctival Fever, Coxsackie virus, Herpangina, and Primary herpetic gingivoestomatitis.