RESUMO
Kikuchi-Fujimoto disease (KFD) is a self-limiting disease of idiopathic origin affecting young women characterized by unexplained fever and lymphadenopathy. It has been usually found to be associated with autoimmunity, of which systemic lupus erythematosus (SLE) is the most outstanding. Fever and lymphadenopathy carry a broad differential, and a missed diagnosis of a rare condition such as Kikuchi can lead to inappropriate treatment in an otherwise benign condition. Therefore, careful examination and histologic confirmation of the diagnosis are critical.
RESUMO
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.