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

Kawasaki disease in Infant/Neonate

See also in: External and Internal Eye,Anogenital,Oral Mucosal Lesion
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Contributors: Tyler Werbel, 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)
For more information, see OMIM.

Codes

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

SNOMEDCT:
75053002 – Kawasaki disease

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Common pitfalls may include diagnosing an infection in an infant with fever and an enlarged cervical lymph node (bacterial lymphadenitis) or sterile pyuria (partially treated urinary tract infection). The subsequent exanthem and mucosal changes may be misdiagnosed as a drug reaction to the prescribed antibiotics.
  • Viral meningitis may be misdiagnosed in an infant with fever, rash, and cerebrospinal fluid pleocytosis.
  • Patients with toxic shock syndrome most often have focal cutaneous skin infections, abscesses, infections associated with nasal packing, or a history of recent surgical procedures.
  • Erythema multiforme presents with symmetrically distributed target lesions. Patients frequently have coexisting herpes orolabialis.
  • Viral exanthems like measles may be difficult to distinguish from KD. However, patients with measles often display an exudative conjunctivitis and Koplik spots within the oral mucosa.
  • The cutaneous and mucosal findings in scarlet fever may also easily be confused with KD. However, these patients respond to anti-streptococcal antibiotic therapy.
  • Staphylococcal scalded skin syndrome presents with erythema and desquamation accentuated within skin folds.
  • Distinguishing drug reactions (eg, Stevens-Johnson syndrome) from KD can be difficult. A careful drug history is critical.
  • KD can present with red cheeks, as does fifth disease (erythema infectiosum). Premature closure on fifth disease leading to missing KD is a diagnostic pitfall.
  • Mycoplasma pneumoniae-induced rash and mucositis (MIRM) – Characterized by prominent mucositis and a sparse eruption that may be polymorphous in association with underlying Mycoplasma pneumoniae infection.
  • Behçet syndrome
  • Contact dermatitis (allergic and irritant)
  • Seborrheic dermatitis
  • Candidiasis
  • Psoriasis
  • Sexual abuse in cases with anogenital involvement

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 08/09/2018
Last Updated: 05/02/2019
Copyright © 2019 VisualDx®. All rights reserved.
Emergency: requires immediate attention
Kawasaki disease in Infant/Neonate
See also in: External and Internal Eye,Anogenital,Oral Mucosal Lesion
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Kawasaki disease : Abdominal pain, Cervical lymphadenopathy, Diarrhea, Fever, Vomiting, Rash, Hand edema, Heart murmur, Irritability, Conjunctival injection, Strawberry tongue
Clinical image of Kawasaki disease
Copyright © 2019 VisualDx®. All rights reserved.