Kawasaki Disease Clinical Presentation

Updated: Jul 29, 2018
  • Author: Tina K Sosa, MD; Chief Editor: Russell W Steele, MD  more...
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Presentation

History

Most children with KD are brought to medical attention because of prolonged fever. There are two forms of KD: complete and incomplete. Diagnosis of complete KD requires fever of at least 5 days' duration along with 4 or 5 of the principal clinical features. The most recent version of the AHA guidelines suggest that if a patient presents with 4 or more of the principal criteria, KD can be diagnosed on day 4 of fever. [2] Experienced clinicians who have treated many KD patients may establish diagnosis before day 4, and patients who present with coronary artery disease can be diagnosed if they have at least 3 of the 5 major diagnostic criteria. [56]  The principal clinical features are as follows:

  • Extremity changes: 1) Erythema of the palms and soles, sometimes accompanied by firm and painful induration of the hands or feet that can impede ambulation 2) Desquamation of the fingers and toes which begins in the periungual region, within 2-3 weeks after the onset of fever 3) Beau's lines (deep transverse grooves across the nails) may be present 1-2 months after fever onset

  • Polymorphous rash: Typically diffuse and maculopapular, but can take on a variety of forms. Bullous, vesicular, and petechial rashes are not consistent with KD. 

  • Oropharyngeal changes: 1) Erythema, fissuring, bleeding, and/or crusting of the lips; 2) Strawberry tongue with prominent fungiform papillae; 3) Diffuse erythema of the oropharyngeal mucosa

  • Bilateral, nonexudative, limbic sparing, painless bulbar conjunctival injection (>90% of patients)

  • Acute unilateral nonpurulent cervical lymphadenopathy with lymph node diameter of at least 1.5 cm (least common criteria present)

Phases of Kawasaki disease

The clinical presentation of KD varies over time, with the clinical course conventionally divided into 3 stages: acute, subacute, and convalescent (see the image below). Some authors add a fourth, chronic, phase.

Clinical manifestations and time course of Kawasak Clinical manifestations and time course of Kawasaki disease.

Stage 1: acutefebrilestage

The acute stage begins with an abrupt onset of fever and lasts approximately 7-14 days. The fever is typically high-spiking and remittent, with peak temperatures ranging from 102-104°F (39-40°C) or higher. This fever is not responsive to antibiotics and can persist for up to 3-4 weeks if untreated. With appropriate therapy with IVIG, the fever typically remits within 36 hours.

In addition to fever and the principal clinical features listed above, signs and symptoms of this phase may include the following:

  • Irritability (often out of proportion to the degree of fever and other symptoms)

  • Aseptic meningitis

  • Anterior uveitis

  • Otitis media

  • Myocarditis and pericarditis

  • Pneumonitis

  • Hepatic, renal, and GI dysfunction

  • Orchitis

  • Meatitis, vulvitis, urethritis

  • Perianal erythema and/or desquamation

Additionally, erythema and induration at the site of bacillus Calmette-Guérin (BCG) inoculation is commonly observed in Japan.

Stage 2: subacute stage

The subacute stage begins when the fevers have abated, and it continues until week 4-6. The hallmarks of this stage are desquamation of the digits, thrombocytosis (the platelet count may exceed 1 million/μL), and the development of CAAs. The risk for sudden death is highest at this stage. 20-40% of patients experience arthralgia or arthritis during this period, both of which tend to favor the large weight-bearing joints. Other characteristics of the subacute stage are persistent irritability, anorexia, and conjunctival injection. 

Persistence of fever beyond 2-3 weeks may be an indication of recrudescent KD. If fever persists, the outcome is less favorable because of a greater risk of cardiac complications.

Stage 3: convalescent phase

The convalescent phase is marked by complete resolution of clinical signs of the illness, usually within 3 months of presentation. This stage begins with the return to baseline of the acute phase reactants and other laboratory abnormalities. Deep transverse grooves across the nails (Beau lines) may become apparent 1-2 months after the onset of fever.

During the convalescent stage, cardiac abnormalities may still be apparent. Smaller CAAs tend to resolve on their own (60% of cases), but larger aneurysms may expand, and MI may occur. In patients whose echocardiograms were previously normal, however, detection of new aneurysms is unusual after week 8 of the illness.

Chronic phase

This stage is of clinical importance only in patients who have developed cardiac complications. Its duration is sometime for a lifetime, because an aneurysm formed in childhood may rupture in adulthood. In some cases of previously undiagnosed aneurysms rupturing in adult life, careful reviews of past medical histories have revealed febrile childhood illnesses of unknown etiology that are suspected to have potentially been unrecognized KD.

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Physical Examination

Because no specific test can be performed for KD and no clinical feature is pathognomonic, the diagnosis of KD is based on the presence of a constellation of clinical findings which may or may not be present at the time of physical exam. [7, 46, 57, 49, 3] ​. This is due to the highly variable presence and time course of the signs and symptoms of KD. This is particularly true for incomplete KD.

Representative photographs of some of the principal clinical features are provided below:

Kawasaki disease: Patchy generalized macular eryth Kawasaki disease: Patchy generalized macular erythema, which is also typical of some viral exanthems.
Kawasaki disease: Peeling and erythema of the fing Kawasaki disease: Peeling and erythema of the fingertips.
Kawasaki disease: Strawberry tongue. Kawasaki disease: Strawberry tongue.

For more information on features of this illness, see the Medscape Reference articles Dermatologic Manifestations of Kawasaki Disease and Ophthalmologic Manifestations of Kawasaki Disease.

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