Kikuchi-Fujimoto Syndrome: A Rare Entity to Consider

INTRODUCTION: Kikuchi-Fujimoto disease (KFD) is a rare, benign, necrotizing lymphadenitis of unknown aetiology with good prognosis. It is characterized by cervical lymphadenopathy, nocturnal diaphoresis and fever. Surgical excision of the adenopathy, histopathological study and immunophenotyping are crucial for diagnosis. PATIENTS AND METHODS: This paper describes five patients with three different histological subtypes of KFD, including an atypical presentation masquerading as pyelonephritis and two other cases where physicians mistakenly started chemotherapy. In one other case cytomegalovirus was identified as the responsible aetiological agent, while in the remaining patient, KFD evolved into an autoimmune condition. DISCUSSION: KFD, although rare, may mimic infectious, autoimmune and neoplastic diseases. It also poses a risk for the subsequent development of an autoimmune disorder. LEARNING POINTS: Kikuchi-Fujimoto disease (KFD), although rare, should be included in the differential diagnosis of patients with cervical lymphadenopathy and fever of unknown origin. Early recognition of KFD may minimize the use of unnecessary aggressive examinations and therapies. The course of KFD in most patients is self-limiting, but there is a risk of progression to an autoimmune syndrome.