Your child feels terribly ill and has a fever for days that is hard to manage, a red tongue, skin rash, swollen lymph nodes and joint pain? A typical childhood disease such as measles or scarlet fever is not always responsible for such symptoms. The rare Kawasaki syndrome also makes itself felt in this way. What is behind it?
What is Kawasaki syndrome?
Kawasaki syndrome (KS) is a life-threatening but rare disease that usually occurs in infancy or childhood. It is one of the vasculitis syndromes, febrile diseases of various causes in which inflammation of the blood vessels is the primary cause. Since the vessels are found throughout the organism, the symptoms are correspondingly varied.
The disease is also known technically as mucocutaneous lymph node syndrome (MCLS).
Kawasaki syndrome: who is affected?
Kawasaki syndrome affects only children (under five years of age in 85 percent of cases), boys more often than girls. Most often, children between one and eight years of age are affected, but adolescents or infants can also become ill.
In Japan, the rate of the disease is many times higher than in other countries. In Germany, an estimated 5 to 17 out of 100,000 children fall ill each year, i.e. about 200 to 600 children per year. Small epidemics are repeatedly reported, occurring particularly frequently in late winter and spring.
Discovery of Kawasaki disease
Kawasaki syndrome owes its name to Japanese physician Tomisaku Kawasaki, who first described it around 1967. Whether the disease actually emerged in the 1960s or existed before the initial description is disputed.
One hypothesis, for example, is that only the introduction of antibiotics made it possible to detect the disease, which had otherwise been hidden behind other symptoms of infection such as scarlet fever. Another conjecture is that the disease is just another course of a form of vascular inflammation (polyarthritis nodosa) – which was also known before.
Interestingly, the same symptoms of the disease were described in Hawaii in the early 1970s, independently of Kawasaki’s publication, which until then existed only in Japanese. Again, researchers still disagree on whether this is coincidence or whether MCLS spread from Japan to the Western world via Hawaii in the late 1960s.
How does Kawasaki syndrome develop?
The exact cause of Kawasaki syndrome is still unknown. However, most experts assume that a pathogen or its toxins (a virus or toxin-producing bacteria) trigger Kawasaki syndrome. Presumably, there must be a hereditary disposition, i.e. a susceptibility of the body to develop corresponding symptoms.
The following factors support this hypothesis, which assumes a combination of infection and genetic predisposition:
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seasonal and geographically clustered occurrence.
- Acute course; symptoms resemble other infectious syndromes caused by bacterial toxins
- Certain genetically determined structures on the cell surface (histocompatibility antigen HLA-Bw22) occur more frequently in affected individuals
It is considered certain that the disease is not contagious and therefore no infection can occur in the family or from other children. However, due to the unclear causes, there is currently also no way to prevent Kawasaki syndrome.
