The Enigmatic Kikuchi-Fujimoto Disease: A Case Report and Review

We report this case of a 33-year-old African American woman who presented to the clinic with preauricular and submandibular masses that she had noticed 6 weeks earlier. She gave a remote history of noticing bilateral cervical masses 3 years prior to this presentation that had not been investigated at the time and resolved spontaneously. Excisional biopsies of the cervical lymph nodes showed morphologic and immunophenotypic findings suggestive of Kikuchi Fujimoto disease (KFD). KFD is an uncommon, self-limited, and perhaps an underdiagnosed entity with an excellent prognosis. It mimics malignant lymphoma in presentation and therefore an accurate clinicopathological differentiation is crucial. 1. Introduction Cervical lymphadenopathy could be a manifestation of a variegated group of illnesses ranging from benign infectious causes to malignant lymphomas. Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing lymphadenitis, was initially described independently by Kikuchi and Fujimoto in 1972. It is a rare, benign, and self-limited syndrome of unknown etiology characterized by tender localized lymphadenopathy, constitutional symptoms such as fever and night sweats [1]. KFD patients are mostly young adults (mean age of diagnosis being 21) [2] with female preponderance (4？:？1) [3–5]. Diagnosis is confirmed histopathologically. It is generally a self-limited disease that regresses spontaneously without any specific therapy. It is an underdiagnosed condition with an excellent prognosis, making it imperative that it is differentiated from a malignant lymphoma. The awareness of this condition amongst clinicians and pathologists alike might help prevent misdiagnosis and inappropriate treatment [1]. This diagnosis should especially be considered in a young patient presenting with cervical lymphadenopathy with biopsy showing necrosis, fragmentation, and karyorrhexis [1, 3, 4, 6]. Recurrence is reported in about 4% of all cases of KFD. We present a case of KFD in a 33-year-old African American woman who had recurrent self-limited episodes of bilateral cervical lymph node enlargement. 2. Case Presentation A 33-year-old African American woman with no medical comorbidities and a recent trip to Jamaica presented to the clinic with complaints of multiple right-sided neck swellings. She reported developing laryngitis while she was in Jamaica, following which she noticed a right preauricular and a right submandibular mass that progressively increased in size. She also complained of fatigue, malaise, and intermittent chills. She had an episode of bilateral