Disease of Kawasaki

The disease of Kawasaki , or lympho-cutanéo-mucous syndrome is a infantile Vascularite feverish concerning the Artère S of means and small gauge of immunological origin , infectious, or para-infectious. It touches the young children (rare beyond five years), and particularly the Asia. It was described in 1967 in Japan where the disease is frequent.

Synonyms

adéno-cutanéo-mucous Syndrome
lympho-cutanéo-mucous Syndrome

Private clinic

clinical Criteria:

Fever of more than five days not yielding to the Antipyrétiques, and not answering the Antibiotic S,
Deterioration of the general state,
bulbar Conjunctivitis bilateral not - exsudative,
Énanthème: framboized language, Chéilite painful with red and fissured lips,
Exanthème Scarlatiniforme, Morbilliforme, also being able to resemble a Syndrome of Stevens-Johnson,
Exanthème with palmo-plantar attack (desquamative erythema Pathognomonic ) at the 4th day, with edema of the dorsal face of the hands and of the feet,
cervical Adenopathy S,
Headaches.

The Desquamation appears towards 2nd or 3rd week of evolution, on the level of the junction between the nail and the pulp of the fingers and the toes.

Biology

There exists a inflammatory Syndrome nonspecific with Hyperleucocytose, VS increased, CRP increased. Sometimes Anemia, Thrombocytose and increase in the IgE.

Diagnosis

The diagnosis rests primarily on the clinical arguments (age, clinical picture and evolution), on the biological inflammatory syndrome and the possible description coronary Anévrisme S with the echography or the Coronarographie.

Evolution

The disease of Kawasaki develops generally favorably into 2 to 3 weeks.

Complication

the risk cardiac with Myocardite, Péricardite and is reached coronary arteries which can involve a infarction, a acute cardiac failure and turbid of the rate/rhythm.
Of the Aneurism S Coronary S, can appear in 15 to 25% of the cases at the desquamative stage , in the absence of treatment, with other lesions of Vascularite.

Differential diagnosis

ictéro-hemorrhagic Leptospirosis;
allergic Eruption ;
Disease of Still (youthful chronic polyarthritis);
polymorphic Erythema but absence of mucous lesions;
Scarlet fever;
Measles;
infectious Mononucleosis;
Collagénose .

Treatment

Aspirine like antiagrégant plate (hyperplaquettose attends)
a single cure of Immunoglobuline S into intravenous with strong amount (2g/kg in 2 days)

Controls

To make an echography of the heart, to measure the dilation of the arteries left and right. for the children whose disease occurred before the five years age, to make a test of effort at 6 years

See too

Erythema

External bonds

Card Kawasaki on orpha.net
Prevenar Vaccine and possible hazard of disease of Kawasaki: reassuring data. (pdf 54 KB) (June 1st, 2006)

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