Weighing Evidence and Art: A Challenging Case of Early-Onset Atypical Kawasaki Disease

Abstract

A 3-month-old previously healthy boy first presented to the emergency department (ED) with a 2-day history of diarrhea, severe dehydration, and fussiness with apneic spells. He also had developed a maculopapular rash on his extremities and trunk that had almost completely resolved on presentation to the ED and had conjunctival erythema with discharge. Several children at home had “pink eye” the same week the infant became ill. From the ED, he was admitted to the PICU, where he was intubated. Although his initial laboratories were unremarkable (white blood cell [WBC] count 11.0 k/uL, 39% neutrophils, platelets 342 k/uL), he also underwent an extensive septic workup (including blood cultures, urine culture, cerebrospinal fluid analysis and culture, herpes simplex virus and enterovirus cultures, respiratory viral panel by polymerase chain reaction, respiratory culture, and influenza A/B direct antigen detection tests) along with empirical treatment with antibiotics for a total of 5 days. These laboratory and imaging studies (serial chest x-rays, head computed tomography scan) were not clinically significant. He defervesced for a 48 hours, but still experienced low-grade fever (Tmax 38.3°C) and intermittent episodes of irritability. After 8 days of hospitalization, he was discharged from the hospital after apparent resolution of most of his symptoms. At his follow-up outpatient pediatric visit 4 days after discharge, 12 days since the onset of his illness, he was afebrile and clinically quiet. ### Question Is there objective evidence that this patient had Kawasaki disease (KD) on initial visit? ### Discussion Tomisaku Kawasaki described the criteria for diagnosing KD in 1967.1 The criteria require presence of fever for duration of ≥5 days along with 4 of the 5 following physical findings without an alternative explanation: