Kikuchi-Fujimoto disease is a form of benign necrotizing lymphadenitis also known as histiocytic necrotizing lymphadenitis. While Kikuchi disease is a globally prevalent disease, it is predominantly seen in the Asian population. Etiology can be traced back to dysregulated immune responses triggered by viral or autoimmune factors. Here we present a case report to emphasize the importance of considering Kikuchi disease and possible etiologies, highlighting the need for increased awareness and recognition of this rare condition. A 28-year- old female with a history of hypothyroidism, IBS, and GERD complained of sore throat, intermittent epigastric pain, mild heartburn, and fever and was taking PPIs without any improvement. Abdominal USG was negative for any pathology. She presented with bilateral enlarged cervical lymph nodes and worsening of symptoms 1.5 months later. Ciprofloxacin was prescribed which failed to improve the symptoms. CT scan of the neck showed an enlarged thyroid gland with diffuse lymphadenopathy. Labs showed elevated TSH, for which the levothyroxine dose was increased, despite that she still continued to have symptoms. LDH and CRP were normal. Rheumatology, oncology, and infectious disease specialists were called upon for further evaluation. Excisional Lymph node biopsy showed areas of extensive necrosis with karyorrhectic debris, surrounded by histiocytes and small lymphocytes, suggestive of necrotizing lymphadenitis, specifically consistent with Kikuchi disease. Immunophenotyping byflow cytometry demonstrated normal CD4+ and CD8+ T cell populations without any evidence of clonal lymphoid expansion. AFB and GMS stains were negative for infection. Hydroxychloroquine 200 mg twice daily was given, which resulted in the resolution of her symptoms. Kikuchi disease’s origins can be traced back to Japan, where it was initially documented. More prevalent in the young female Asian population under the age of 30. It presents as tender, enlarged nodes, primarily in the cervical region along with lymphadenopathy, fever, erythematous rash, joint pain, fatigue, arthritis, and hepatosplenomegaly. It becomes important to consider Kikuchi disease in patients presenting with symptoms that can be attributed to sore throat related to GERD, followed by the development of fever with cervical lymph node enlargement.

Keywords : Kikuchi-Fujimoto disease, benign necrotizing lymphadenitis, Asian population, dysregulated immune response, cervical lymphadenopathy.