Aims To describe clinical characteristics of childhood Kikuchi Disease (KD) so as to increase awareness among clinicians; this would hopefully lead to early recognition and appropriate management. We also sought to determine risk factors for recurrent KD.

Methods This was a retrospective review of children <16 years-of-age with histologically proven KD from 2005 to 2012. We retrieved clinical records and collected data on clinical characteristics, laboratory findings, treatment and outcomes. Chi-Square analysis was performed to determine risk factors for recurrent KD.

Results Fifty-two patients were identified; 56% (29/52) were male, with a median age of 11.1 years (92.3% were older than 7 years-of-age). Thirty-one children (59.6%) presented with fever, with a median duration of 10 days. At presentation, 24 children (46.1%) had complaints of fever and neck swelling, 21 had neck swelling alone, while 7 presented only with fever. Median duration of neck swelling at presentation was 28 days. Despite this, all cases had prominent cervical lymphadenopathy detected on physical examination.

Two children (3.8%) had a history of Kawasaki disease, 2 had lymphoma/ leukaemia previously requiring chemotherapy, and 2 had a family history of Systemic Lupus Erythematosus (SLE). Five (9.6%) had a recent history of cervical lymphadenitis. Out of 32 children tested for antinuclear antibody (ANA) titres, 5 were positive; only 1 had a titre >1/800.

None of the patients responded to antibiotic therapy, and all required complete excision biopsy, with a median time to diagnosis of 9.5 days. Sixteen patients (30.8%) did not respond to complete excision and required adjunctive steroid therapy. Median duration of steroid therapy was 16 days.

Median duration of follow-up was 1 year. Six children (11%) had recurrent KD. The only risk factor identified for recurrent KD was fever at initial presentation (p = 0.032). One patient, who was diagnosed with KD at 5 years of age, subsequently developed SLE at age 15 (he was ANA negative throughout presentation and subsequent follow-up).

Conclusion Kikuchi Disease should be considered in children with subacute cervical lymphadenopathy with or without prolonged fever. These children are at risk of developing subsequent SLE and long-term follow-up is required.