Kikuchi-Fujimoto disease

Key words: Kikuchi-Fujimoto disease, Histiocytic necrotizing lymphadenitis, Autoimmune disorders, Case report

Kikuchi-Fujimoto disease (KF), also known as histiocytic necrotizing lymphadenitis, is a rare benign condition characterized by cervical lymphadenopathy, prolonged fever and other systemic manifestations without specific laboratory findings. The etiology is unknown but an exaggerated immune response to an autoimmune or viral trigger is hypothesized. It can mimic malignancies such as lymphoma; the diagnosis is based on the histological examination of a lymph node biopsy. There is no specific treatment for KF, but symptomatic therapy is indicated. In severe cases corticosteroids or other immunomodulating agents have been used. Recurrences are possible and a risk of development of autoimmune disorders (primarily SLE) is described. The paper reports the unusual case of an 8-year-old Caucasian girl with relapsing KF (3 episodes) that presented with cervical and supraclavicular lymphadenopathy. The importance of a correct diagnosis and long-term follow-up is emphasized.