Kikuchi-Fujimoto disease: A comprehensive review

Core Tip

Core Tip: Kikuchi-Fujimoto disease or Kikuchi disease described originally in young Japanese women is a rare benign cause of fever and lymphadenopathy usually involving cervical lymph nodes. The disease has been reported worldwide in both genders across ethnic and age groups. Histopathologically, histiocytic necrotizing lymphadenitis is characteristic and needs differentiation from more serious conditions such as malignant lymphoma in acute or subacute form. Its long-term prognosis is favorable albeit long-term follow-up is recommended because of a rare but increased risk of developing systemic lupus erythematosus/other autoimmune disorders. Patients with a severe or recurrent disease need treatment with nonsteroidal anti-inflammatory drugs, systemic corticosteroids, and/or other immunomodulators.