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Kawasaki Syndrome

Kawasaki’s disease is a disease of unknown etiology. The manifestations relate to super-antigen formation and it is a multi-system inflammatory disease. And the main thing is fever of five days or more and then four of the remaining five manifestations Kawasaki's syndrome, Kawasakis disease, Kawasaki syndrome. The definition is a surveillance definition and I think one of the take-home messages is that you should be prepared, in young children.

There are certain things that just seeing it alone, and that is changes in the extremities, edema, induration of the hands and feet, is enough to strongly suspect Kawasaki’s disease. Other findings are conjunctival injection, mouth lesions, fissuring, crusting of the lips, strawberry tongue. The induration of the hands and feet in a child under a year is diagnostic in itself. Rash of quite variability and the least common manifestation is enlarged lymph nodes, even thought the original description included lymph nodes in the title. Associated with this is evidence of multi-system pyuria, various other findings. Important is aseptic meningitis and this should help you with the diagnosis, not turn you away from the diagnosis.

This is a picture and the rash can be multiforme, it can be just erythematous maculopapular, more important thing here is the swelling of the hands. The scleral conjunctivitis. This is late in the illness, the desquamation in the tips of the fingers and this is desquamation in the feet. This is the red palm, one of the original papers from Japan. Strawberry tongue in a Kawasaki’s patient. Now the crucial thing here, or the main problem with Kawasaki’s disease, are giant aneurysms of multiple different arteries, and the important ones are coronary arteries leading to infarcts. So patients need to be treated and you need to make a decision. With fever for a few days you have a lot of differentials of which Kawasaki’s is sort of at the bottom. Once you get beyond five days there are less differential possibilities.

Treatment is IVIG, and the most usual treatment today is 2 gm/kg over 12 hours. Also for inflammation, aspirin in high dose, four doses a day at 100 mg/kg. Then later put the patient on long-term aspirin.

There are differences in this but certainly you need to wait until evidence of acute inflammation is over. There is no evidence that aspirin has any benefit as far as the heart is concerned, but there is evidence that IVIG treatment is beneficial. The other thing is that one dose is frequently not enough, so roughly 25% of patients need one or more doses.

Next is toxic shock syndrome and this is also a staphylococcal toxin and sometimes a streptococcal toxin. This is similar to the erythrogenic toxin of scarlet fever and very different from exfoliative toxin from phage group II staph. Here the classic disease of course was the epidemic relating to the staphylococcal infections with tampons, which the primary source went unrecognized and the manifestations were the toxic shock. It was actually described first in young children by Jim Todd relating to localized infections with disseminated toxin, usually skin infections. The manifestations; hypotension, clinical or laboratory abnormalities in greater than or equal to three organ systems, and reasonable evidence of ruling out other etiologies.

Okay, the last of the diseases that have some similarity with scarlet fever is erythema infectiosum and this is caused by parvovirus B19. It has a case-to-case interval of 6-14 days. The important thing is that after exposure about a week later you are contagious, but generally asymptomatic or have a mild fever. Then a week after that the rash occurs and at that time the patient is no longer contagious. The rash starts on the face with a "slapped cheek" appearance. The original rash starts centrally, spreads peripherally, is not very diagnostic, but takes on a lace-like pattern. The rash is more prominent on extensive surfaces and adults have arthralgias and arthritis. This is a classic picture from almost 100 years ago, showing the slapped cheek appearance.

Now I want to switch over and talk a bit about enteroviruses, and I’m going to say more at the end about enteroviruses. But a lot of clinical manifestations can occur with enterovirus infections. This is … almost 40 years ago I occupied my time studying these things so everybody yawns when I try to make a big deal out of this. So I’m just going to go through and give you some major manifestations of what you should be aware of. Then we’ll come back and talk specifically about enterovirus epidemiology. I’m going to talk about EcHO-9 coxsackie A-9 and coxsackie A-16 as examples. EcHO virus 9 was the first of the enteroviruses to be well characterized with exanthems, with exanthem and aseptic meningitis.