Kawasaki disease M30.3

Kawasaki 1967

Probably immunologically mediated, diffuse vasculitis (vasculitis of medium-sized vessels) occurring predominantly in childhood, which is clinically characterized by high fever, enlarged cervical lymph nodes, skin and mucous membrane infestation and in 15-25% of cases is complicated by the addition of myocarditis and coronariitis with consecutive thrombus and aneurysm formation in the coronary vessels.

Rare disease, highest incidence in Japan (4-6,000 diseases/year or 140/100,000/year). In Southeast Asia the incidence in children <5 years is 112/100,000 per year, in Europe 8.1/100,000 per year and in Germany approximately 8/100,000 per year.

Unexplained, pathogen-related triggering is likely (e.g. SARS-Cov-19). There is also a genetic predisposition. Change in the activity of a C-kinase that intervenes in the activation of T-lymphocytes (1,4,5-triphosphatase-3-kinase C).

It has been shown on several occasions that Kawasaki syndrome and Kawasaki-like syndrome can be associated with COVID-19 and that the symptoms overlap. Children with COVID-19 may present as Kawasaki-like disease with pediatric inflammatory multisystem syndrome or macrophage activation syndrome (Wang L et al. 2021).

Furthermore, various susceptibility genes are known to play a role in the pathogenesis of the disease: ITPKC, CASP3, CD40 and ORAI(Onouchi Y 2018).

Mostly occurring in infants aged 1-5 years, rarely in young adults. Slight emphasis on the male sex.

General: High, antibiotic-resistant fever lasting longer than 5 days. Conjunctivitis (>75%). Painful, cervical lymph node swelling (25-75%), pharyngitis (90%)

Integument: bright red, chapped lips, diffuse reddening of the oral and pharyngeal mucosa, possibly raspberry tongue (90%). Palmoplantar erythema (50-85%) with edematous transformation (50-85%); after 2-3 weeks desquamation, which begins in a crescent shape on the fingertips. Later Beau-Reil's transverse furrows of the fingernails.

Polymorphic exanthema: morbilli- or scarlatiniform, erythema exsudativum multiforme-like(70-90%).

Other accompanying symptoms: gastroenteritis, urethritis, abacterial meningitis, arthralgias, gallbladder hydrops.

Reactivation of the BCG vaccination site in the form of redness and induration is a further indication of the disease (however, BCG is no longer a standard vaccination in Germany).

Cardiac symptoms, which can occur 12-28 days after infection, are a complication. They manifest themselves in coronaritis and can lead to (giant) aneurysms, thrombosis, arrhythmias, stenosis, myocardial infarction, heart failure and PAD.

Leukocyturia and proteinuria, pronounced leukocytosis with left-shift, CRP and alpha-2-globulins increased Thrombocytosis from the 2nd week of illness.

Scarlet fe ver: Clinical picture, pathogen detection, antistreptolysin titer (AST) rise (8-14 days after fresh infection at the earliest, baseline and control values), Dick test, extinction phenomenon.

Measles: Clinical picture with typical exanthema and enanthema (palate, tonsils, uvula). Titer increase by 2 levels in the haemagglutination inhibition test.

Infectious mononucleosis: generalized lymphadenopathy, splenomegaly, morbilliform exanthema, + serology.

Brucellosis; Weil 's disease: rare

Multisystemic inflammatory syndrome in association with COVID-19 (evidence of a previous or still active COVID-19 disease)

Acute rheumatic fever: carditis, polyarthritis (migratory), chorea minor (Sydenham), erythema marginatum rheumaticum (formerly also eythema anulare rheumaticum), and subcutaneous nodules (rheumatoid nodules). Secondary criteria: fever, arthralgias.

Hand-foot-and-mouth disease: fever, usually no severe feeling of illness, painful vesicles.

Still's syndrome: long-term course with recurrent fevers.

Erythema exsudativum multiforme: typical clinical morphology of the exanthema with cocards

Multisystemic inflammatory syndrome associated with COVID-19, often a severe disease in children and adolescents. The phenotype resembles Kawasaki syndrome. Dermatological findings include severe febrile general symptoms, maculopapular exanthema, concunctivitis, cheilites, palmar erythema, and hand and foot edema.

Early diagnosis and therapy is important.

Untreated, the disease can heal completely within 12-28 days.

The frequent (dreaded) cardiac complications occur 12-28 days after the onset of the disease.

The lethality rate is about 1-2%, mainly due to heart attacks.

Decisive for the course and prognosis is the extent of vascular involvement.

Therapy recommendations for Kawasaki syndrome (modified according to Cremer)

Worldwide, 2019 coronavirus disease (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) appeared to have a milder clinical course in children compared with adults. European and US pediatricians increasingly noticed cases of myocarditis sharing some clinical features with toxic shock syndrome, Kawasaki syndrome, and macrophage activation syndrome in otherwise healthy patients (Berardicurti O et al. 2020). The spectrum of severity ranged from standard hospitalization to treatment in the pediatric intensive care unit (Ebina-Shibuya R et al. 2020). The term multisystemic inflammatory syndrome was introduced for this new Kawasaki-like syndrome in associationCOVID-19 .

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