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Kikuchi-Fujimoto Syndrome: A Rare Entity to Consider.

Diogo Raposo André1, Filipa Vicente1, Jessica Chaves1

  • 1Internal Medicine Department, Hospital Central do Funchal, Madeira Island, Portugal.

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Summary

Kikuchi-Fujimoto disease (KFD) is a rare lymphadenitis presenting with fever and swollen lymph nodes. Early diagnosis is key to avoid misdiagnosis and aggressive treatments, as KFD can mimic other serious conditions.

Keywords:
Kikuchi-Fujimoto diseasefever of unknown originlymphadenitisrare disease

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Area of Science:

  • Pathology
  • Immunology
  • Infectious Diseases

Background:

  • Kikuchi-Fujimoto disease (KFD) is a rare, benign necrotizing lymphadenitis of unknown cause.
  • It typically presents with cervical lymphadenopathy, fever, and nocturnal diaphoresis.
  • Diagnosis relies on histopathological study and immunophenotyping of lymph node biopsies.

Purpose of the Study:

  • To highlight the diverse clinical presentations of KFD.
  • To emphasize the importance of considering KFD in the differential diagnosis of fever of unknown origin.
  • To discuss the potential for KFD to evolve into autoimmune disorders.

Main Methods:

  • Case series describing five patients with KFD.
  • Inclusion of patients with atypical presentations and varied etiologies.
  • Review of diagnostic challenges and treatment implications.

Main Results:

  • KFD can present atypically, mimicking conditions like pyelonephritis.
  • Misdiagnosis leading to unnecessary chemotherapy occurred in some cases.
  • Cytomegalovirus was identified as a potential etiological agent in one case.
  • One patient developed an autoimmune condition following KFD.

Conclusions:

  • KFD is a rare condition that can mimic infectious, autoimmune, and neoplastic diseases.
  • It requires inclusion in the differential diagnosis for unexplained cervical lymphadenopathy and fever.
  • While often self-limiting, KFD carries a risk of subsequent autoimmune syndrome development.