Kikuchi-Fujimoto disease: a rare but important cause of lymphadenopathy

Cervical lymphadenopathy is a common problem in children. Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis) is a rare condition that causes persistent lymphadenopathy and has a characteristic histological appearance. Kikuchi-Fujimoto disease is well recognized in Japan, where it was first described, but descriptions in the paediatric literature are sparse. Paediatricians may therefore be unaware of this rare but important condition. The case is described of a 14-y-old girl suffering from fatigue, weight loss, night sweats and lymphadenopathy. The predominance of systemic symptoms prompted an extensive investigation. The diagnosis of Kikuchi-Fujimoto disease was made after direct histological examination of a lymph node biopsy. The case illustrates the clinical features of this little-known condition and highlights the potential confusion with other diagnoses. The pathological features are discussed and the literature reviewed.

Kikuchi-Fujimoto disease should be considered in cases of persistent lymphadenopathy. An early biopsy can be instrumental in preventing unnecessary investigations and potentially harmful treatments.