What is Kawasaki Disease (skin and mucosal syndrome)?
Kawasaki disease (BD) is a systemic acute disease of predominantly newborns and young children, characterized by persistent fever, bilateral conjunctivitis, lesions of the mucous membranes of the mouth and lips, skin of the extremities, exanthema on the body and acute non-suppurative lymphadenitis. Described for the first time T. Kawasaki et al. as a new childhood disease-febrile mucousto-lymphatic iron syndrome.
BC is prevalent mainly in Japan, where several thousand cases are registered, and to a lesser extent in the USA. In Japan, the average age of patients is 1 year, in the USA – 3.8 years. Boys get sick more often than girls.
It is characterized by symptoms of panangiitis of the coronary arteries with lymphohistiocytic infiltration, ruptures of the elastic membrane, areas of necrosis and the formation of aneurysms. Pronounced panangiitis is also found in the iliac arteries, less often in other large vessels. At the same time, endocarditis, myocarditis, lymphadenitis with necrosis can be detected.
Causes of Kawasaki Disease (skin and mucosal syndrome)
The association of CD with infectious factors (rickettsiae, Epstein-Barr virus, streptococci) is assumed.
Pathogenesis during Kawasaki Disease (mucous skin-iron syndrome)
The pathogenesis is not well understood, but predominantly obvious is the immune genesis, which is confirmed, in particular, by the detection of deposits of immune complexes in the affected tissues.
Symptoms of Kawasaki Disease (mucous skin-iron syndrome)
BC, like childhood infections, is very dynamic. In the first week of the disease, fever, redness of the palms and soles, thick edema of the legs, papular rash, disappearing by the end of the 1st week of the disease appear. At the same time, conjunctivitis, erythematous reddening of the lips and oral mucosa, and cervical lymphadenitis develop. On the 2nd week all clinical signs of the disease disappear, body temperature returns to normal. However, in a number of patients, fever may last for several weeks. A special feature of fever is antibiotic resistance.
Already in the acute phase of the disease, pancarditis may develop with affection of the mitral and, more rarely, aortic valves. In severe disease, heart failure, coronary artery aneurysms, and myocardial infarction are observed. Of the other clinical manifestations of systemic disease – diarrhea, articular syndrome, CNS damage (aseptic meningitis, encephalopathy), etc.
Diagnosis of Kawasaki Disease (mucous skin-iron syndrome)
Laboratory data. In the acute phase of the disease, leukocytosis is observed with a shift to the left, an increase in ESR.
Diagnosis. The disease is mainly diagnosed with characteristic signs that change over time. In recognizing the nature of cardiac pathology, ECG, coronary angiography and echocardiography are of great importance.
Differential diagnosis. CD is differentiated from childhood infections (scarlet fever and meningococcemia, viral infections), as well as from vasculitis, DBST, JRA, Stevens-Johnson syndrome, etc. As rightly pointed out by R. R. Cupps and AS Fauci, a differential diagnosis of CD can be difficult to develop. cardiac pathology, since it manifests itself during the subsidence of the acute symptoms of the disease.
Treatment of Kawasaki Disease (mucostokin and ferrous syndrome)
In most cases, in the acute period of the disease, symptomatic therapy is prescribed, including nonsteroidal anti-inflammatory therapy. Antibiotics are ineffective. The question of the feasibility of treatment of GCS has not yet been resolved.
The prognosis is usually favorable in 98-99% of cases, only 1-2% die with symptoms of acute heart failure during the development of myocardial infarction or rupture of a coronary artery aneurysm.