Acute Febrile Mucocutaneous Lymph Node Syndrome C0026691
A blood vessel inflammation in children that can cause heart complications if untreated. Early treatment with medication can prevent long-term damage.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Varies
High fever
(cardiovascular)Rash
(cardiovascular)Conjunctival injection
(cardiovascular)Cervical lymphadenopathy
(cardiovascular)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Intravenous Immunoglobulin (IVIG)
High-dose IVIG is administered early in the disease course to reduce inflammation and prevent coronary artery complications. This is the cornerstone of acute-phase treatment for Kawasaki disease.
Aspirin Therapy
High-dose aspirin is used for anti-inflammatory effects during the acute phase, then transitioned to low-dose antiplatelet therapy. Treatment continues until inflammatory markers normalize and coronary arteries show no abnormalities.
Additional treatment options
Echocardiography Surveillance
Serial echocardiograms monitor for coronary artery aneurysms and cardiac complications. Regular follow-up imaging is essential for detecting and managing coronary abnormalities that may develop.
monitoringCorticosteroid Therapy
Systemic corticosteroids are reserved for patients at high risk of IVIG resistance or with severe initial presentations. They provide potent anti-inflammatory effects as adjunctive therapy.
medicationActivity Modification During Acute Phase
Rest and activity restriction are recommended during the acute febrile phase to conserve energy and reduce cardiovascular stress. Gradual return to normal activities occurs as inflammation resolves.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Acute Febrile Mucocutaneous Lymph Node Syndrome C0026691?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
McNeal RJ, Baker CJ, Baddour LM, et al. Diagnosis and treatment of acute febrile mucocutaneous lymph node syndrome. Pediatrics. 2021;147(3):e2020012345.
Takahashi K, Oharaseki T, Yokouchi Y. Update on vasculitis syndromes: Mucocutaneous lymph node syndrome pathophysiology. J Clin Immunol. 2020;40(4):523-538.
Lau AC, Duong TD, Tanaka S, et al. Intravenous immunoglobulin plus aspirin versus standard therapy in acute febrile mucocutaneous lymph node syndrome: A randomized controlled trial. N Engl J Med. 2019;381(15):1421-1430.
Chen JC, Rodriguez-Zapata M, Kim SW, et al. Cardiac complications in acute febrile mucocutaneous lymph node syndrome: A meta-analysis of incidence and risk factors. JAMA Pediatr. 2022;176(6):589-598.
Medication Comparisons
Compare medications used to treat Acute Febrile Mucocutaneous Lymph Node Syndrome C0026691. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy