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4A44.5 Mucocutaneous lymph node syndrome

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

Mucocutaneous lymph node syndrome (Kawasaki disease) is a globally distributed acute vasculitis of young children affecting medium to small calibre arteries and if untreated leads to coronary artery aneurysms in about a quarter of cases. Cardinal signs include cervical lymphadenopathy, conjunctival injection, rash (maculopapular, erythrodermic or erythema multiforme-like), strawberry tongue, oropharyngeal erythema, erythema and swelling of hands and feet and periungual desquamation. The cause is unknown but thought to be environmental, possibly from viral infection. A variant of the disease has been linked to SARS CoV-2 infection.

inclusions

• Kawasaki syndrome

synonyms

• Mucocutaneous lymph node syndrome
• Acute febrile mucocutaneous lymph node syndrome
• Kawasaki syndrome
• Kawasaki disease

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