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

Kawasaki disease - Oral Mucosal Lesion

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

Synopsis

Kawasaki disease (KD), or mucocutaneous lymph node syndrome, is a multisystem vasculitis that affects infants and children. The disease occurs primarily in children under the age of 6. It is classically characterized by fever lasting at least 5 days (usually longer) plus 4 of the 5 following criteria:
  • Conjunctival injection without exudate
  • Red lesions of the mouth or pharynx
  • Acute hand and foot edema followed by peeling
  • Polymorphous cutaneous eruption
  • Lymphadenopathy (usually cervical)
Oral mucous membrane changes are present in roughly 90% of patients with KD. The most common findings are diffuse oral mucositis, erythematous tongue with prominent fungiform papillae ("strawberry tongue"), and cracked lips. Only one of these findings is necessary to fulfill the diagnostic criteria. Discrete oral lesions, such as vesicles or ulcers, and exudative inflammation are not consistent with KD.

Most individuals affected by KD follow a benign disease course. However, coronary artery aneurysms develop in 20%-25% of untreated patients. Treatment is aimed at decreasing the risk of developing coronary artery abnormalities and preventing complications from these abnormalities.

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

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • 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.
  • 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.
  • 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.
  • For oral findings, also consider hand-foot-and-mouth disease, stomatitis (contact, aphthous), and candidiasis.

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:08/08/2018
Last Updated:02/16/2022
Copyright © 2023 VisualDx®. All rights reserved.
Emergency: requires immediate attention
Kawasaki disease - Oral Mucosal Lesion
See also in: Overview,External and Internal Eye,Anogenital
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.
Copyright © 2023 VisualDx®. All rights reserved.