Recurrent Kikuchi-Fujimoto Disease Masquerading as Lymphoma Successfully Treated by Anakinra

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare and benign disease that usually presents in middle-aged women of Oriental-Asian ethnicity. This condition was described in Japan for the first time in 1972. Though the clinical course is benign, KFD has been misdiagnosed as malignancy (e.g. lymphoma) or infection. The most common presentation of KFD is with localized or generalized lymphadenopathy, fever, fatigue, weight loss, hepatosplenomegaly, and rash. A definitive diagnosis of KFD can be made by excisional lymph node biopsy, as immunohistochemical analysis is necessary. We present here an interesting case of a 20-year-old Hispanic female who was diagnosed with KFD who failed therapy with steroids and was subsequently treated successfully with the interleukin-1 (IL-1) inhibitor - anakinra.