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Kikuchi-Fujimoto disease: A comprehensive review.

Vikram K Mahajan1, Vikas Sharma2, Neeraj Sharma2

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Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting lymph node disorder affecting young adults. Diagnosis requires careful histopathology, as KFD can mimic other conditions like lymphoma and lupus.

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Cervical lymphadenopathyHistiocytic necrotizing lymphadenitisKikuchi’s diseaseLymphadenopathyLymphomaSystemic lupus erythematosus

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Area of Science:

  • Immunology
  • Pathology
  • Rheumatology

Background:

  • Kikuchi-Fujimoto disease (KFD) is a rare, benign, self-limiting necrotizing lymphadenitis of unknown cause.
  • It primarily affects young adults, presenting with fever and lymphadenopathy, and can have cutaneous manifestations.
  • KFD shares a complex relationship with systemic lupus erythematosus (SLE) and is often misdiagnosed.

Purpose of the Study:

  • To review the clinicoepidemiological, diagnostic, and management aspects of KFD.
  • To highlight the diagnostic challenges and differential diagnoses of KFD.
  • To provide insights for practicing clinicians managing KFD.

Main Methods:

  • Review of clinicoepidemiological data.
  • Analysis of diagnostic modalities, including histopathology.
  • Evaluation of current management strategies.

Main Results:

  • KFD presents with fever and lymphadenopathy, with variable cutaneous involvement in 30-40% of cases.
  • Differential diagnoses include lymphoma, SLE, and infectious lymphadenitis.
  • Fine needle aspiration cytology is often nonspecific; histopathology is diagnostic.

Conclusions:

  • Accurate diagnosis of KFD relies on careful histopathological evaluation of lymph node biopsies.
  • Early biopsy is crucial to avoid unnecessary investigations and empirical treatments.
  • Management, often empirical, may involve corticosteroids or hydroxychloroquine.