Mucocutaneous lymph node syndrome [Kawasaki]

This code represents Kawasaki disease, an acute febrile illness primarily affecting young children, characterized by inflammation of blood vessels throughout the body, particularly the coronary arteries. It is a form of vasculitis that can lead to serious cardiac complications if not promptly diagnosed and treated.

Use this code for confirmed cases of Kawasaki disease, whether acute, subacute, or convalescent stages. Documentation should clearly state the diagnosis of Kawasaki disease, often supported by clinical criteria such as fever, conjunctivitis, oral mucosal changes, rash, and lymphadenopathy.

• Do not use this code for other forms of vasculitis; ensure the documentation specifically identifies Kawasaki disease.
• Code any associated cardiac complications, such as coronary artery aneurysm, separately and sequence them appropriately.
• Ensure the medical record distinguishes between acute, subacute, and chronic phases if relevant for treatment or follow-up.