A 19 year old with Fever, rash, and conjunctivitis: a connection with the heart.

The patient was a 19-year-old African American male who presented to the hospital with complaints of fever, diffuse maculopapular rash (chest, bilateral upper extremities, and back), bilateral conjunctivitis, and abdominal pain. His symptoms began approximately 3 weeks prior to presentation to our hospital. Over the 3-week period he developed a constellation of symptoms to include a polymorphous eruption, right facial nerve palsy, and bilateral conjunctivitis (Figure 1). Before presenting to our hospital, he had multiple evaluations that included near normal evaluation including a normal head computed tomography (CT) scan, mildly elevated liver-associated enzymes (LAEs), negative human immunodeficiency virus, and syphilis serologies. On presentation to our facility he was febrile to 38.3°C, and his exam was notable for right facial palsy, edematous lips, and enlarged erythematous tongue with raised papilla (Figure 1). He also exhibited a mild leukocytosis, sterile pyuria, and elevated lipase and erythrocyte sedimentation rate (ESR) (Table 1). He was admitted for further evaluation and management. Infection, drug reaction, and rheumatologic process were all considered in the differential, and the various studies (laboratory tests, radiographs, electrocardiogram, and transthoracic echocardiogram [TTE]) performed to evaluate for these causes did not identify a clear etiology (Table 1). His abdominal pain, facial nerve palsy, and edematous lips and tongue improved with supportive care, and he was discharged with plans for follow-up examination. He was readmitted 6 days later with continued temperatures to 38.0°C (despite scheduled acetaminophen) and continued nonpurulent conjunctivitis. In addition, he now also had new severe bilateral lower extremity pain, swelling of bilateral ankle and feet, and desquamation of his palms and soles with notable periungual desquamation (Figure 1). This constellation of symptoms was suggestive of Kawasaki’s disease (KD). Laboratory markers were also supportive of the diagnosis leukocytosis (13.8 × 10/μL), normocytic anemia (11 g/dL), thrombocytosis (535 × 10/μL), elevated markers of inflammation (ESR, 94 mm/hour and C-reactive protein, 18 mg/dL), and elevated LAEs (alanine aminotransferase, 111 U/L and aspartate aminotransferase, 62 U/L). The second day of this admission, he developed complaints of dull substernal chest pain. A repeat TTE was performed and compared to one obtained a week prior. A new left anterior descending coronary artery dilatation and ectatic vessels were noted (Figure 2). Given the coronary artery changes, treatment for KD was deemed necessary. The patient was given intravenously administered immunoglobulin (2 mg/kg) and high-dose aspirin (50 mg/kg per day). As is typical with pediatric patients, our patient defervesced 1 day after initiation of therapy with dramatic overall clinical improvement. After 3 days, he was subsequently transitioned to 81 mg of aspirin per day. He had continued follow-up tests with CT angiography performed 4 months later, which showed normal coronary arteries without any evidence of aneurysmal changes. At that time, aspirin was discontinued and the patient was advised to arrange follow-up visits with the cardiology department annually. KD is a systemic vasculitis of unknown etiology that usually affects infants and children, with 76% of the cases in children in the United States occurring in individuals below the age of 5 [1]. Adult cases are far less reported, with approximately 100 cases reported in Received 1 December 2014; accepted 2 January 2015. Correspondence: Tida Lee, MD, PhD, Infectious Disease Fellow, Walter Reed National Military Medical Center, Infectious Diseases Clinic, Bldg 7, 1st Fl, Bethesda, MD 20889 (tida.k.lee.mil@mail.mil). Open Forum Infectious Diseases Published by Oxford University Press on behalf of the Infectious Diseases Society of America 2015. This work is written by (a) US Government employee(s) and is in the public domain in the US. DOI: 10.1093/ofid/ofv008