Kawasaki disease is also known as mucocutaneous lymph node syndrome. It is a systemic, medium-sized vessel vasculitis. It affects young children, typically under 5 years. There is no clear cause or trigger. It is more common in Asian children, particularly Japanese and Korean children. It is also more common in boys. A key complication is coronary artery aneurysm.
Clinical Features
A key feature that should make you consider Kawasaki disease is a persistent high fever (above 39ºC) for more than 5 days. Children will be unhappy and unwell. The key skin findings are a widespread erythematous maculopapular rash and desquamation (skin peeling) on the palms and soles.
Other features include:
- Strawberry tongue (red tongue with large papillae)
- Cracked lips
- Cervical lymphadenopathy
- Bilateral conjunctivitis
TOM TIP: If you come across a child with a fever persisting for more than 5 days, think of Kawasaki disease! A rash, strawberry tongue, lymphadenopathy and conjunctivitis will seal the diagnosis in your exams.
Investigations
There are several investigations that can be helpful in Kawasaki disease:
- Full blood count can show anaemia, leukocytosis and thrombocytosis
- Liver function tests can show hypoalbuminemia and elevated liver enzymes
- Inflammatory markers (particularly ESR) are raised
- Urinalysis can show raised white blood cells without infection
- Echocardiogram can demonstrate coronary artery pathology
Disease Course
There are three phases to Kawasaki disease:
- Acute phase: The child is most unwell with the fever, rash and lymphadenopathy. This lasts 1 – 2 weeks.
- Subacute phase: The acute symptoms settle, the desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming. This lasts 2 – 4 weeks.
- Convalescent stage: The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. This last 2 – 4 weeks.
Management
There are two first line medical treatments given to patients with Kawasaki disease:
- High dose aspirin to reduce the risk of thrombosis
- IV immunoglobulins to reduce the risk of coronary artery aneurysms
Patients will need close follow up with echocardiograms to monitor for evidence of coronary artery aneurysms.
TOM TIP: Kawasaki disease is one of the few scenarios where aspirin is used in children. Aspirin is usually avoided due to the risk of Reye’s syndrome. This is a unique fact that examiners like to test.
Last updated January 2020