Acute febrile mucocutaneous lymph node syndrome [MCLS]

This code represents Kawasaki disease, an acute, self-limiting vasculitis primarily affecting young children. It is characterized by fever, rash, conjunctivitis, oral mucosal changes, lymphadenopathy, and extremity changes, with a significant risk of coronary artery aneurysms if untreated.

Use this code for documented cases of Kawasaki disease, also known as mucocutaneous lymph node syndrome (MCLS). Documentation should clearly indicate the presence of the characteristic clinical criteria for diagnosis, typically including prolonged fever and at least four of the five principal clinical features.

• Ensure documentation specifies "acute" to differentiate from sequelae.
• Code any associated complications, such as coronary artery aneurysms, separately and sequence appropriately.
• Do not confuse with other febrile illnesses or rashes; specific diagnostic criteria for Kawasaki disease must be met.