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Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenitis seen mostly in Asian populations (Kikuchi in Nippon Ketsueki Gakkai Zasshi 35:379-80, 1972). First described in 1972, KFD is a benign and self-limiting disease characterised by lymphadenopathy, mild fever, fatigue, and leukopenia (Fujimoto in Naika 30:920-7, 1972; Lin et al. in Otolaryngol Head Neck Surg 128(5): 650-3, 2003). We present a case of a 38-year-old woman with a 6-week history of cervical lymphadenopathy. Her ultrasound scan and fine needle aspiration cytology results were inconclusive. Excisional biopsy of the lymph node confirmed presence of KFD. The aetiology of KFD is unknown; however, there is strong association with systemic lupus erythematosus (SLE). Kucukardali reported 9% of European KFD patients and 28% of East Asian patients had concomitant SLE (Kucukardali et al. in Clin Rheumatol 26(1):50-4, 2007). We describe a follow-up algorithm for newly diagnosed KFD cases, based on the current literature. KFD is a rare cause of cervical lymphadenopathy. It is associated with increased risk of developing SLE, therefore early diagnosis and long-term follow-up are recommended.

Kikuchi–Fujimoto Disease and Prognostic Implications