AN UNUSUAL CHALLENGING CASE OF CERVICAL LYMPHADENITIS -KIKUCHI FUJIMOTO DISEASE

Abstract

Introduction: Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign, and self-limiting clinicopathological entity first described independently by Kikuchi and Fujimoto in Japan in 1972. It predominantly affects young women and is characterized by cervical lymphadenopathy, fever, and constitutional symptoms. Although the disease is generally self-limiting, its clinical presentation often mimics more serious conditions such as tuberculous lymphadenitis, lymphoma, metastatic lymphadenopathy, and systemic lupus erythematosus (SLE), leading to diagnostic challenges. Histopathological examination remains the cornerstone for establishing a definitive diagnosis.

Case Presentation: We report a rare case of Kikuchi-Fujimoto disease in a 20-year-old female with no significant past medical history who presented with acute onset painful left-sided cervical lymphadenopathy associated with malaise, intermittent fever reaching 100°F, and significant weight loss. Clinical examination revealed multiple enlarged cervical lymph nodes on the left side of the neck, with the largest node measuring approximately 2 × 3 cm. Fine-needle aspiration cytology suggested necrotizing lymphadenitis, demonstrating irregularly shaped pale areas composed predominantly of histiocytes and plasmacytoid dendritic cells with absence of neutrophils. In view of persistent cervical lymphadenopathy, the patient underwent excision biopsy under general anaesthesia. Intraoperatively, multiple enlarged lymph nodes involving cervical levels II, III, IV, and V were identified and excised. Histopathological examination revealed characteristic features of Kikuchi-Fujimoto disease, including patchy areas of necrosis with abundant karyorrhectic nuclear debris and absence of neutrophilic infiltration. Immunohistochemical analysis further confirmed the diagnosis. Following definitive diagnosis, the patient was treated with systemic corticosteroids and demonstrated a dramatic clinical improvement with complete resolution of symptoms.

Conclusion: Kikuchi-Fujimoto disease should be considered as an important differential diagnosis in young patients presenting with unilateral cervical lymphadenopathy accompanied by constitutional symptoms. Early recognition and histopathological confirmation are essential to avoid unnecessary investigations and inappropriate treatment. Furthermore, patients diagnosed with KFD should undergo long-term follow-up because of its documented association with systemic lupus erythematosus.