Kikuchi Fujimoto Disease – A Diagnostic Challenge

Kikuchi-Fujimoto Disease is an extremely rare entity characterized by subacute necrotizing lymphadenitis and frequently associated with fever. It is known to have a worldwide distribution with higher prevalence among Japanese and other Asiatic young individuals. The clinical, histopathological and immunohistochemical features appear to point to a viral etiology, a hypothesis that still has not been proven and the cause remains uncertain. It is generally diagnosed on the basis of an excisional biopsy of affected lymphnodes. Early diagnosis with excisional lymph node biopsy is crucial as this disease can be mistaken for Systemic Lupus Erythematosus (SLE) or lymphoma and to avoid unnecessary investigations. The treatment is only symptomatic unless complicated, where steroid therapy is considered. Kikuchi’s disease has an excellent prognosis with almost no risk. Because of the rarity and difficulty in diagnosis, we thought of publishing the case.