Kawasaki disease

Mucocutaneus lymph node syndrome otherwise known as Kawasaki disease is a Vasculitis unknown etiologic. It causes multisystem inflammatory disease targeting small and medium size arteries, leading to a creation of aneurysms.The disease targets mainly children up to the age of 5 with a maximum between second and third year of age. The highest incidence is in Japan. Asian children are affected 6 times more often than Caucasian ones. In the Czech Republic the incidence is guessed to be 1.6/100 000 children up to 5 years.

Acute phase

 * Sudden fevers (over 40°C);
 * bilateral non-purulent conjunctivitis;
 * changes to mucous (dry, cracked lips, erythema of buccal mucosa and strawberry tongue);
 * cervical Lymphadenopathy (more than 1,5cm in diameter) – in 70% of cases ;
 * erythema and tough swelling of hands and feet + swelling of PIP hand joints ;
 * polymorphic exanthem mostly in inguinal region and on thorax- in 80% of cases ;
 * symptoms of acute myocarditis (sinus tachycardia, gallop rhythm, weak heart sounds) ;
 * other: stomach pains, gallbladder hydrops, pleocytosis in CSF, arthritis (middle size and large size joints);
 * lasts 1–2 weeks.

Subacute phase

 * Desquamation of skin (mainly of fingers);
 * thrombocytosis(as much as 1012/liter) ;
 * creation of aneurysms of coronary arteries - risk of Sudden death;
 * risk factors: prolonged fevers, prolonged elevation of inflammation markers, age under 1year, male sex;
 * lasts till the fourth week.

Recovery phase

 * From disappearance of clinical symptoms to decline of inflammation markers to normal (usually 6.-8. weeks from the first symptoms);
 * the Beau's lines can appear on nails during this phase.

Diagnostics

 * Exclude other causes of fever (infection);
 * Hemoculture, cultivation of urine, thorax X-RAY;
 * heightened inflammation markers, thrombocytosis;
 * Lumbar puncture (to exclude infection) – pleocytosis;
 * echo – evidence of aneurysms of coronary arteries.

Diagnostic criteria

 * Fever lasting longer than 5 days and minimally 4 of the following:
 * bilateral non-purulent conjunctivitis;
 * reddened, dry cracked lips, raspberry tongue, …;
 * peripheral erythema, peripheral edema, pealing of skin on fingers, generalized desquamation;
 * polymorphic exanthema on the abdomen, the thorax and the area of genitals;
 * cervical lymphadenopathy (over 1,5 cm in average).

Treatment

 * i. v. imunoglobulin (IVIG);
 * Acetylsalicylic acid (Aspirin) in anti-inflammation dose (80–100mg/kg/den) in acute phase and in antiaggregating dose (3–5mg/kg/den) in other phases.

Related articles

 * Systemic vasculitis