Kikuchi-Fujimoto Disease Is a Rare Cause of Lymphadenopathy and Fever of Unknown Origin in Children

Abstract

Kikuchi-Fujimoto disease, a benign and unusual self-limiting histiocytic necrotizing lymphadenitis of unknown origin, should be included in the differential diagnosis of lymphadenopathy and fever of unknown origin. This disease mostly affects young Asian women and has rarely been reported in children, thus remaining a poorly recognized entity that is frequently confused with malignant lymphoma. The authors describe two children with Kikuchi-Fujimoto disease, with particular attention to diagnostic approach and clinical and histologic features of the disease.

Unusual Cause of Prolonged Fever of Unknown Origin

“…Common presenting symptoms are fever and cervical lymphadenopathy. 1,3 While extranodal involvement is rare, there have been reports of cases with presenting symptoms of skin lesions, 4,5 arthritis, 5,6 and, as with our case, aseptic meningitis 5 and weight loss. 7 Unfortunately, no specific laboratory tests contribute to the diagnosis of KFD, 3,8 though the most commonly reported laboratory findings seen in KFD are leukopenia 1,3,9 and elevated lactate dehydrogenase 1 and ESR.…”

“…Numerous infectious pathogens have been postulated to be causative agents of KFD including parasite, viruses, and bacteria, though none have been confirmed. 4 It has also been proposed that KFD is the result of an autoimmune reaction due to its similarities to SLE in laboratory findings and clinical presentation. 10 There have been several case reports of KFD being associated with SLE, while others have speculated that KFD may lead to SLE later in life.…”

Fever of unknown origin (FUO) caused by Kikuchi's disease mimicking lymphoma

“…Findings include lymphadenopathy, hepatosplenomegaly, leukopenia, elevated erythrocyte sedimentation rate, and increased liver enzymes. [1][2][3][4][5] There have been rare reports of Kikuchi's disease presenting as FUO. [6][7][8][9][10][11] The differential diagnosis includes all causes of infectious mononucleosis/infectious mono-like illnesses and malignancies, particularly lymphomas.…”

Fever without apparent source on clinical examination

“…Scagni et al [24] describe two children with KikuchiFujimoto disease (KFD) as a rare cause of lymphadenopathy and fever of unknown origin. KFD is a rare, benign, self-limiting histiocytic necrotizing lymphadenitis of unknown origin and should be considered in the differential diagnosis of lymphadenopathy and fever of unknown origin.…”