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Emergency: requires immediate attention
Kawasaki disease in Infant/Neonate
See also in: External and Internal Eye,Anogenital,Oral Mucosal Lesion
Other Resources UpToDate PubMed
Emergency: requires immediate attention

Kawasaki disease in Infant/Neonate

See also in: External and Internal Eye,Anogenital,Oral Mucosal Lesion
Contributors: Tyler Werbel MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Kawasaki disease (KD), or mucocutaneous lymph node syndrome, is an idiopathic, multisystem vasculitis characterized by fever and mucocutaneous inflammation. It has a peak incidence in infants aged 9-11 months and is extremely rare in infants younger than 3 months of age. Most cases occur in individuals who live in East Asia or are of Asian ancestry. Although usually self-limited, potentially life-threatening coronary artery aneurysms may develop in 20%-25% of children without treatment (versus less than 5% with appropriate therapy). Mortality most often occurs within the first weeks to a year after KD due to ischemic heart disease caused by myointimal proliferation within persistent aneurysms. Infants younger than 6 months of age may be at increased risk for aneurysms.

The classic case definition of KD is fever lasting at least 5 days plus the presence of at least 4 of the following principal clinical criteria:
  • Bilateral bulbar conjunctival injection without exudate
  • Oral mucosa changes: cracked lips, "strawberry tongue," or diffuse erythema of the mucosae
  • Changes in the extremities: erythema, induration, or periungual peeling
  • Exanthem
  • Cervical lymphadenopathy (greater than 1.5 cm diameter)
A multisystem inflammatory syndrome potentially linked to COVID-19 has been reported in children and young adults; clinical features include Kawasaki-like and toxic shock syndrome-like presentations.

Codes

ICD10CM:
M30.3 – Mucocutaneous lymph node syndrome [Kawasaki]

SNOMEDCT:
75053002 – Kawasaki disease

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Last Reviewed:08/08/2018
Last Updated:01/18/2022
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Emergency: requires immediate attention
Patient Information for Kawasaki disease in Infant/Neonate
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Emergency: requires immediate attention
Kawasaki disease in Infant/Neonate
See also in: External and Internal Eye,Anogenital,Oral Mucosal Lesion
A medical illustration showing key findings of Kawasaki disease : Abdominal pain, Cervical lymphadenopathy, Diarrhea, Fever, Vomiting, Rash, Hand edema, Heart murmur, Irritability, Conjunctival injection, Strawberry tongue
Clinical image of Kawasaki disease - imageId=266240. Click to open in gallery.  caption: 'Erosions and hemorrhagic crusting on the lips.'
Erosions and hemorrhagic crusting on the lips.
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