Kikuchi-Fujimoto Disease and Associated Lymphadenopathies

Kikuchi-Fujimoto disease (KFD) is a rare, benign form of histiocytic necrotizing lymphadenitis predominantly affecting young adults, with a slight female preponderance. Clinically, it presents with tender cervical lymphadenopathy, fever, night sweats and, less frequently, weight loss or rash. Histopathological examination reveals necrotic foci surrounded by CD8+ T lymphocytes and histiocytes, without neutrophil infiltration. Although the precise aetiology remains uncertain, accumulating evidence suggests a dual contribution of viral triggers and dysregulated immune responses, including interferon production and plasmacytoid dendritic cell activation. Distinction from systemic lupus erythematosus, lymphoma and infectious lymphadenitides is critical to avoid unnecessary interventions. Most cases resolve spontaneously within one to four months, although corticosteroids may be used in severe or prolonged presentations. A subset of patients experience recurrence or progression towards autoimmune sequelae, underscoring the need for long-term follow-up. Recent advances have refined diagnostic algorithms, clarified risk factors for relapse and introduced subtyping based on clinical and laboratory features, enhancing prognostic stratification and guiding management.

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Technical terms

Lymphadenopathy: Enlargement of lymph nodes due to infection, inflammation or malignancy.

Necrotizing histiocytic lymphadenitis: A pathological pattern characterised by coagulative necrosis within lymph nodes accompanied by histiocyte infiltration and absence of neutrophils.

Antinuclear antibody (ANA): Autoantibodies targeting components of the cell nucleus, often associated with autoimmune diseases.

Complement component 4 (C4): A protein of the classical complement pathway that participates in immune complex clearance and inflammation.

Plasmacytoid dendritic cells: A specialised dendritic cell subset that produces large amounts of type I interferons in response to viral stimuli.

References

1. Case report: Kikuchi-Fujimoto disease: unveiling a case of recurrent fever and enlarged cervical lymph nodes in a young female patient with a literature review of the immune mechanism. Frontiers in Immunology (2024).
2. Kikuchi‐Fujimoto disease: investigating comprehensive clinicopathological features and risk factors for recurrence. Histopathology (2025).
3. Symptom-based Kikuchi disease subtypes: Clinical scenarios across specialties in Taiwan with temporal trends analysis. Journal of Microbiology Immunology and Infection (2025).
4. Kikuchi-Fujimoto disease. Orphanet Journal of Rare Diseases (2006).
5. Diagnosis of Kikuchi-Fujimoto Disease: A Comparison between Open Biopsy and Minimally Invasive Ultrasound-Guided Core Biopsy. PLOS ONE (2014).