History and exam
Key diagnostic factors
common
polymorphous rash
Nonspecific polymorphic rash. This is usually a diffuse, maculopapular erythematous rash. Occasionally scarlatiniform- or erythema multiforme-type rash with target lesions on the arm and trunk.
Groin erythema or desquamation and fine pustules over extensor surfaces of extremities can occur.
conjunctival injection
The patient has a history or presents with nonpurulent nonexudative bilateral conjunctival injection (in 90% of cases).
Less common are episcleritis or uveitis (anterior and/or posterior).
mucositis
History or physical findings of dry, erythematous, fissured lips that bleed easily, erythema of the oral and pharyngeal mucosa, and strawberry tongue with prominent papillae and erythema (alone or in combination in 90% of cases). Oral exudation or Koplik spots are not present and oral ulceration would be atypical. Strawberry tongue may be present, but oropharyngeal/mucosal changes may be variable.
skin changes in the peripheral extremities
Initial erythema and edema may be present, usually of palms and soles. Typically the skin on wrists and ankles would not be involved. Peripheral changes may be acute (swelling and erythema) as well as subacute (desquamation).
Periungual desquamation of fingers and toes about 2 weeks after onset may be seen, as may transverse grooves across the nails (Beau lines) 1 to 2 months after onset.
enlarged cervical lymph nodes
Unilateral lymphadenopathy is observed in approximately 40% of patients, with node diameter above 1.5 cm.
The overlying skin may be erythematous. The node is not fluctuant or purulent and is unresponsive to antibiotics.
coronary artery aneurysms
Coronary artery abnormalities (mainly aneurysms) develop in approximately 20% to 25% of untreated patients.
fever and extreme irritability
Fever usually over 102°F (39°C). Most patients present because of prolonged fever of at least 5 days in duration with often abrupt onset. Fever is unresponsive to antibiotics, if given. Patients are often irritable beyond that expected for the extent of fever.
There is an associated significant irritability. During this acute phase, many patients will develop poor intake, abdominal pain, nausea, and diarrhea.
Other diagnostic factors
uncommon
pericarditis with effusion
Not part of the diagnostic criteria.
congestive heart failure
Not part of the diagnostic criteria.
joint pain or edema
Arthralgia and arthritis involving multiple joints (e.g., including hands, knees, ankles, and hips) are more common if intravenous immune globulin treatment is delayed.
neurologic manifestations
Headaches and stiff neck (secondary to aseptic meningitis), facial palsy, and cerebral infarction are rare, but can occur.
gastrointestinal manifestations
Abdominal pain, vomiting, pseudo-obstruction, diarrhea, hepatitis, obstructive jaundice, gallbladder distension or hydrops of the gallbladder, and pancreatitis are rare clinical findings. Gastrointestinal symptoms may precede typical Kawasaki disease symptoms.[35]
urologic manifestations
Sterile pyuria is the most common, but meatitis, urethritis and vulvitis (in females), proteinuria, nephritis, and acute renal failure can occur.
other dermatologic manifestations
Peripheral extremity gangrene, pustules, erythema multiforme-like lesions, perianal desquamation, macules, papules, measles-like rash, and scarlet-fever-like erythema are rare clinical findings.
Risk factors
strong
Asian ancestry
KD is most common in Asian children, especially those of Japanese descent.
Some cases of KD show familial susceptibility. Children in Japan who have parents with KD seem to have a more severe form of this disease and are more susceptible to recurrence.
KD likely has a genetic susceptibility. Genome-wide multipoint linkage analysis of affected sibling pairs in Japan identified evidence of linkage at chromosome 12q24.[27]
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