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Pathology

Affects medium sized vessels, skin, mucous membrane and lymph nodes. Potentially fatal affect on coronary arteries in children.

Aetiology

Commonest between 6 months to 4 years, peak incidence at 12 months, more common in Japanese patients, often following viral infection .

Signs

‘Strawberry tongue’, perineal rash, erythema/desquamation of palms, cervical lymphadenopathy, Beau’s lines

Symptoms

High, prolonged fever not responsive to Paracetamol, bilateral non-exudative conjunctivitis, sore mouth, arthralgia, systemic upset

Investigations

Bloods: ESR, CRP
Imaging: ECG and Echo to screen for cardiac complications

Treatment

Medical: Immunoglobulin within 10 days lowers risk of coronary aneurysms
6 weeks Aspirin or longer if abnormal Echo to reduce thrombosis risk
Surgical: Coronary aneurysm repair if required

Prognosis

Good with early treatment
Coronary artery aneurysms 

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Key References

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