Kikuchi-Fujimoto Disease: A Rare Lymphadenitis with Diagnostic Challenges and Treatment Considerations

Abstract

Kikuchi-Fujimoto disease (KFD) is a rare form of lymphadenitis that primarily affects young women in Japan and other parts of Asia. It is caused by unknown agents, possibly viruses that trigger an autoimmune response in genetically susceptible individuals. KFD is often misdiagnosed due to its resemblance to other benign and malignant conditions, including SLE-related lymphadenitis and lymphoma. Diagnosis of KFD requires a lymph node biopsy, which reveals characteristic features such as histiocytic necrotizing lymphadenitis. Additional diagnostic tests, including skin biopsy, MRI, and cerebrospinal fluid analysis, can aid in confirming the diagnosis. KFD is self-limiting, and most patients recover within a few months with symptomatic treatment. However, early intervention with corticosteroids and hydroxychloroquine is crucial for effective management, particularly in cases with extra-nodal involvement or neurological symptoms. Lymph node removal can also be an effective therapeutic measure, and Hemophagocytic lymphohistiocytosis (HLH), a potential complication of KFD, is managed with a combination of immunoglobulins and steroids. While KFD is a benign condition, its diagnosis and treatment can be challenging, requiring close monitoring and follow-up.