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Kikuchi-Fujimoto Disease

Summary

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  • Rare, self-limiting condition characterised by cervical lymphadenopathy and fever
  • Benign histiocytic necrotising lymphadenitis with unknown aetiology
  • Diagnosis based on clinical presentation, lymph node biopsy, and exclusion of other causes

Pathophysiology

  • Exact cause unknown, but likely an autoimmune response to an infectious trigger
  • Proposed mechanisms:
    • Viral infection (e.g., Epstein-Barr virus, human herpesvirus 6)
    • Autoimmune disorder (association with systemic lupus erythematosus)
  • Histopathology shows:
    • Paracortical necrosis with karyorrhectic debris
    • Histiocytic infiltrate with absence of neutrophils
    • Proliferation of CD68+ histiocytes and CD8+ T-lymphocytes

Demographics

  • Predominantly affects young adults (20-30 years old)
  • Female preponderance (female to male ratio 4:1)
  • More common in Asian populations, but can occur in any ethnic group
  • Rare in children and elderly

Diagnosis

  • Clinical presentation:
    • Cervical lymphadenopathy (unilateral or bilateral)
    • Fever
    • Night sweats
    • Fatigue
    • Weight loss
  • Laboratory findings:
    • Leukopenia
    • Elevated erythrocyte sedimentation rate (ESR)
    • Elevated C-reactive protein (CRP)
  • Definitive diagnosis:
    • Excisional lymph node biopsy with characteristic histopathological findings
  • Differential diagnosis:
    • Lymphoma
    • Tuberculosis
    • Systemic lupus erythematosus
    • Cat scratch disease
    • Toxoplasmosis

Imaging

  • Ultrasonography:
    • Enlarged hypoechoic lymph nodes
    • Preservation of hilar vascularity
    • Cortical thickening
  • Computed Tomography (CT):
    • Multiple enlarged lymph nodes, typically in cervical region
    • Homogeneous enhancement
    • Absence of necrosis or extracapsular spread
  • Magnetic Resonance Imaging (MRI):
    • T1: Isointense to muscle
    • T2: Hyperintense
    • Post-contrast: Homogeneous enhancement
    • Diffusion-weighted imaging: Restricted diffusion
  • 18F-FDG PET/CT:
    • Increased FDG uptake in affected lymph nodes
    • Useful for excluding malignancy and monitoring treatment response

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Treatment

  • Generally self-limiting, resolving within 1-4 months
  • Supportive care:
    • Analgesics
    • Antipyretics
  • Severe or persistent cases:
    • Corticosteroids (e.g., prednisolone)
    • Hydroxychloroquine
  • Rare cases with recurrence or progression:
    • Immunosuppressive therapy (e.g., cyclosporine)
  • Regular follow-up to monitor for:
    • Resolution of symptoms
    • Development of autoimmune disorders (e.g., systemic lupus erythematosus)

Differential diagnosis

Differential Diagnosis Differentiating Feature
Lymphoma Kikuchi-Fujimoto Disease (KFD) lacks atypical lymphocytes and has characteristic histopathological findings
Tuberculosis KFD does not show caseous necrosis or positive acid-fast bacilli staining
Cat Scratch Disease KFD lacks the history of cat exposure and the characteristic stellate abscesses
Sarcoidosis KFD does not show non-caseating granulomas typical of sarcoidosis
Hodgkin's Lymphoma KFD lacks Reed-Sternberg cells and the typical immunophenotype of Hodgkin's lymphoma