Kikuchi Disease in Korean Children: Clinical Features and Disease Courses.

OBJECTIVES: Kikuchi’s disease (KD) is a benign, self-limiting disorder of unknown origin with lymphadenopathy and fever, and prevalent in young Asian women. The initial presentations mimic malignancies or other serious infections, and the biopsy of lymph nodes affected is the only definite diagnostic tool of KD. We analyzed data from a series of pediatric cases collected at a single medical center and suggest proper guideline of management for patients with KD.

METHODS: The medical records of children under 16 years of age, who underwent lymph node biopsies and received histiopathologic diagnosis of KD at Ajou University Hospital, South Korea from January 2000 to June 2007, were retrospectively reviewed.

RESULTS: Twenty six pediatric patients with KD included 10 boys and 16 girls. In all cases except two, the cervical lymph nodes were involved. Twenty (76.9%) patients had fever as a presenting symptom, and 5 patients (19.2%) had various skin rash. Leukopenia was noted in 8 cases (30.8%), but there was no case of leukocytosis. The levels of ESR and LDH were slightly elevated, but CRP and liver enzymes were within normal range. No KD patients in this study developed any other autoimmune disorders during follow-up period. Eleven (55%) cases with fever improved immediately after excision biopsy of affected lymph nodes. Eight (30.8%) cases treated with steroid showed dramatic responses. Six patients (23.1%) had resolutions of symptoms simply with symptomatic treatments. The recurrence happened in 6 (23.1%) children within a few weeks from the first episode.

CONCLUSIONS: Contrary to previous reports on Asian cases, KD in Korean children is not rare in young boys, and fever is more common presenting symptoms. It’s relationship with autoimmune diseases or infection of EBV is not clear. Symptoms such as fever improved in some cases only by the removal of affected lymph node for excision biopsy. In agreement with previous reports, patients treated with steroid recovered dramatically. Therefore, we suggest an earlier excision biopsy for patients suspicious with KD and an immediate treatment of cases with severe symptoms with steroid. The recurrence rate in this study was higher than that in previous studies, therefore, a spell of follow-up period after disappearance of symptoms is advisable for patients with original diagnosis of KD.