ABSTRACT

Kawasaki disease is a vasculitis of infant and childhood period. Diagnosis of Kawasaki disease is based on clinical criteria including cervical lymphadenopathy. Here we reported a 3.5-year-old boy presenting with Kawasaki disease mimicking bacterial lymphadenitis and fever. If a child presents with lymphadenopathy only which is unresponsive to antibiotics, Kawasaki disease should be considered.

Introduction

Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome and infantile polyarteritis nodosa was first described in 1967 by Tomisaku Kawasaki (1,2). The disease most frequently occurs in children aged between 6 months and 5 years. KD is the leading cause of pediatric acquired heart disease in developing countries. There is no specific diagnostic test for KD. The diagnosis is made based on the presence of clinical criteria. Typical KD criteria include fever going on for more than 5 days, cervical adenopathy, nonpurulent bilateral conjunctival injection, oral mucosal changes, hand-foot changes in extremities and polymorfic rash on body. Four of 5 clinical criteria define diagnosis (3). Of the principal diagnostic criteria, cervical adenopathy is the least common. However, KD manifests with fever and cervical adenopathy and may be misdiagnosed as bacterial cervical lymphadenitis. Therefore, specific treatment is delayed, leading to serious cardiac sequelae.

Herein, we report an unusual case of KD: a 3.5-year-old boy who presented with fever and bilateral cervical lymphadenitis as initial manifestations.

Case Report

A 3.5-year-old boy presented with a high fever, swelling in the neck, fatigue, restlessness and dehydration. He had a history of persistent fever for ten days, reaching 40.5 ⁰C, especially at nights. Before admission, he received an oral treatment of amoxicillin-clavulanate 90 mg/kg/24 hr for 4 days ceftriaxone 80 mg/kg/24 hr, and two doses of metronidazole 30 mg/kg/24 hr parenterally. Physical examination revealed three lymph node enlargements unilaterally in cervical region, the largest being, 30 mm x 30 mm in diameter; fixed, solitary, tender without fluctuation. Non-specific complaints were present including abdominal cramping, weight loss due to decreased oral intake and change in defecation. Blood testing showed white blood cell (WBC): 14.5000/mm3 with neutrophil predominance (55%); hemoglobin: 10.4 g/dL; platelet counts: 291.000/mm3, C-reactive protein (CRP): 7.5 mg/dL, and sedimentation ratio: 66 mm/h. Biochemical markers were normal except for slightly reduced albumin (2.9 g/dL). Soft-tissue neck ultrasonography revealed multiple lymph nodes, of fusiform shaped-reactive nature, of which the biggest was located at the right juguler triangle, 30x12 mm in diameter. Neck computerized tomography was obtained to exclude deep cervical infection. Multiple and diffuse lymphadenitis were seen at the level of superficial and deep cervical lymph nodes. The biggest one was on the right side, at jugulodigastric localization, 32x16x20 mm in diameter (Figure 1). Treatment with intravenous clindamycin 30 mg/kg/24 hr was initiated to treat possible infectious components, hydration was administered via inravenous route, but his condition did not improve, and the neck masses remained painful. The patient exhibited with enlarged cervical nodes three days after admission. The throat swab culture and blood culture samples taken at the time of admission were negative for infection. Incomplete Kawasaki syndrome was suspected due to the child’s irritated, tired appearance; continuing fever, and bilateral cervical lymphadenitis an echocardiography was performed showing increased thickness of coronary artery walls (Figure 2). The patient was diagnosed with KD based on the echocardiographic findings. Immediate treatment with intravenous immnunoglobulin (2 g/kg for one day) and 80 mg/kg/day asetilesalysalic acid was initiated. Significant improvement was observed on the first day after the treatment. The lymph node sizes and acute phase response were reduced on the following day. Besides, the patient’s fever subsided and the other symptoms resolved. Control laboratory analyses revealed: WBC counts 9650/mm3, platelet counts 691.000/mm3, sedimentation ratio 30 mm/h, and CRP 0.3 mg/dL. Follow-up echocardiography examinations after one week were normal. On the 9th day of the treatment, desquamation on the patient’s fingers and toes, and macular rash on his feet were observed. Two weeks later, asetilsalysalic acid treatment dose was decreased to antiaggregant level, and the patient was discharged. Follow-up echocardiography examinations during the 2-month period after discharge were normal.

Discussion

KD is a systemic vasculitis affecting small and medium-sized vessels and is most often seen in children between 6 months and 5 years (4). It’s etiology is still unknown but clinical, laboratory and epidemiological features suggest an infectious origin (5). Typical Kawasaki symptoms include fever- of more than 5 days-being unresponsive to antibiotic treatment, cervical lymphadenitis, nonpurulent bilateral conjuctival injection, oral mucosal changes, hand-foot changes in the body. In KD, lymph node enlargements are less frequent than the other findings (25-50%) (6), and their incidence is more frequent in patients of older ages. Lymphadenopathy and fever have previously been reported as initial presentations of KD in the literature. Additionaly KD has sometimes mimicked bacterial lymphadenitis by including prominent swelling, pain, and redness with high body temperatures. This may cause a delay in the diagnosis of KD and increase the risk of coronary artery involvement. Nomura et al. (7) evaluated laboratory and clinical findings of 187 patients with confirmed KD and 16 patients presented with only fever and cervical lymphadenopathy on admission. Three of sixteen patients presented with bacterial cervical lymphadenitis or deep neck infection (7). Kubota et al. (8) reported that patients with KD presenting with only fever and cervical lymphadenopathy were older and admitted earlier. Another case report by April et al. (9) demonstrated that patients with lymphadenopathy were older and had stronger inflammatory responses than those without lymphadenopathy. Yap et al. (10) reported a 10 year-old boy, admitted with acute exudative tonsillitis and bilateral cervical lymphadenitis. The patient had a 4-day history of fever, sore throat, neck pain and an unresponsive fever despite 72 hours of intravenous antibiotic treatment. Fourteen days post admission the patient presented sheet-like desquamation of the hands, feet, and perianal region. KD was diagnosed mimicking bacterial lymphadenitis and fever. They suggested that children presenting with sole fever and cervical lymphadenopathy could be considered as having KD.

In our case we tried to emphasize that KD can present as bacterial lymphadenitis. In case of acute bacterial lymphadenitis unresponsive to antibiotic regimen, KD should be considered in differential diagnosis.