Kikuchi-Fujimoto disease (KFD) is characterized by lymphadenopathy and fever, and is usually self-limited. This study analyzed the clinical characteristics of pediatric patients with KFD.

This retrospective, observational, single-center study was conducted in South Korea from March 2008 to October 2015. KFD was diagnosed based on clinical, radiological or histological findings and excluded when there were any other causes of lymphadenopathy. Medical records were reviewed for clinical and laboratory manifestations.

A total of 35 cases were included. The mean patient age was 12.1±2.9 years (range, 5 to 17 years); the male-to-female ratio was 1:0.8. The main clinical manifestations were cervical lymphadenopathy and fever in 34 cases (97%). The mean duration of fever was 12.2±8.3 days (range, 2 to 37 days). We noted enlargement of lymph nodes in the cervical, mesenteric (n=5, 14%), axillary (n=2, 6%), and inguinal (n=1, 3%) regions. Hepatosplenomegaly, loss of appetite, and rash were observed. On laboratory examinations, elevation of ferritin, leukopenia, and positivity for anti-nuclear antibodies were frequently observed. Twelve patients underwent biopsy and 23 cases were diagnosed by radiological findings. The mean duration of hospitalization for all cases was 7.9±2.9 days (range, 3 to 13 days) and steroids were administered in 10 cases. KFD recurrence was observed in 2 cases (5.7%) with the time to relapse of 7 months and 4 years. There were no cases with systemic lupus erythematous or other autoimmune disease.

KFD should be considered in pediatric patients with lymphadenopathy and prolonged fever. Patients with KFD should be monitored for recurrence and the development of autoimmune disease.