Case Presentation:

A 27‐day‐old Asian female infant was admitted for a fever of 102°F and a subsequent neonatal sepsis workup. A CBC with differential, blood, urine, and CSF cultures was performed. She was started on ampicillin and cefotaxime. All labs and cultures were normal. On hospital day 2 she developed a maculopapular rash over her body excluding palms and soles. She later developed cracked lips and bilateral, nonexudative conjunctival injection. On day 3, she developed a diaper rash associated with peeling of skin around the perineal area. Mild edema of the hands and feet was noticed. She continued to spike temperatures to 103° F despite being on antibiotics and remained fussy. A repeat lumbar puncture was performed to test for herpes, and acyclovir was added and later discontinued after HSV PCR was negative. After a review of her symptoms and consultation with a pediatric ID specialist, she was treated for Kawasaki's disease (KD) with IVIG and high‐dose aspirin. Her temperature de‐fervesced and her rash improved within 24 hours. A heart echocardiogram done on day 3 of hospitalization was read as normal. A repeat echo in 2 weeks showed dilation of proximal coronary arteries. Her platelet count on admission was normal and rose to 1 million on day 14 of the illness. The infant was sent home on low‐dose aspirin and was scheduled to have follow‐up with cardiology with a final diagnosis of KD.


Kawasaki disease (KD) is an acute, self‐limited generalized vasculitis of unknown cause that has a striking predilection for the coronary arteries of infants and young children. Recent investigations revealed that KD has an oligoclonal and IgA driven immune response. Young infants may present with atypical KD, with fever the only presenting symptom. These patients are at higher risk of developing coronary abnormalities. Echocardiography should be performed as soon as KD is suspected and again 2 and 6 weeks after disease onset. Supportive laboratory evidence suggestive of systemic inflammation, for example, anemia, elevated acute‐phase reactants, hypoalbuminemia, sterile pyuria, aseptic meningitis, may facilitate making the diagnosis. Early treatment with IVIG and high‐dose aspirin, preferably within 10 days of onset of symptoms, helps to reduce coronary abnormalities by 85%.


KD is rare in young infants, with the median age at presentation being 2 years. Prompt recognition of atypical features of KD in young infants is especially important, as early treatment helps to reduce the incidence of coronary artery abnormalities and thus fatal outcomes.

Author Disclosure:

R. Kamat, none; P. Jain, none; J. Robertson, none.