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

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)
Codes
ICD10CM:M30.3 – Mucocutaneous lymph node syndrome [Kawasaki]
SNOMEDCT:
75053002 – Kawasaki disease
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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.
- Multisystem inflammatory syndrome in children (MIS-C)
- 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.
- COVID-19
- 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.
- Reactive infectious mucocutaneous eruption (RIME) – Characterized by prominent mucositis and a sparse eruption that may be polymorphous in association with underlying Mycoplasma pneumoniae or other infection.
- Behçet syndrome
- Contact dermatitis (allergic and irritant)
- Seborrheic dermatitis
- Candidiasis
- Psoriasis
- Sexual abuse in cases with anogenital involvement
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References
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Last Reviewed:08/08/2018
Last Updated:12/11/2022
Last Updated:12/11/2022
Emergency: requires immediate attention
Kawasaki disease in Infant/Neonate
See also in: External and Internal Eye,Anogenital,Oral Mucosal Lesion