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Kikuchi-Fujimoto disease: a clinicopathologic update.

F Pepe1, S Disma1, C Teodoro1

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Summary

Kikuchi-Fujmoto disease (KFD) is a rare, self-resolving lymphadenitis typically presenting as painful cervical lymphadenopathy. Diagnosis relies on lymph node biopsy, distinguishing it from lymphoma and autoimmune disorders.

Keywords:
Cervical adenopathyFeverHistiocytic necrotizing lymphadenitisKikuchi-Fujimoto disease

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Area of Science:

  • Pathology
  • Immunology
  • Oncology

Background:

  • Kikuchi-Fujmoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a rare, benign lymph node disorder of unknown etiology.
  • It predominantly affects young adults, often presenting with cervical lymphadenopathy and constitutional symptoms, and typically resolves spontaneously within months.

Purpose of the Study:

  • To summarize the key clinical, histological, and diagnostic features of Kikuchi-Fujmoto disease.
  • To highlight the importance of early and accurate diagnosis to avoid unnecessary investigations for malignancy or autoimmune conditions.

Main Methods:

  • Review of clinical presentations, diagnostic criteria, and histological findings associated with KFD.
  • Discussion of differential diagnoses, including systemic lupus erythematosus and lymphoma.
  • Emphasis on histopathological examination of lymph node biopsies for definitive diagnosis.

Main Results:

  • KFD is characterized by cervical lymphadenopathy, fever, and constitutional symptoms, with a favorable prognosis and spontaneous resolution.
  • Histological examination reveals paracortical necrosis with characteristic immune cell infiltrates (histiocytes, plasmacytoid dendritic cells, CD8+ lymphocytes).
  • Absence of neutrophils, plasma cells, and hematoxylin bodies aids in differentiating KFD from SLE-related lymphadenopathy.

Conclusions:

  • Accurate diagnosis of KFD is essential, primarily based on lymph node biopsy findings.
  • Distinguishing KFD from lymphoma and autoimmune diseases like SLE is crucial for appropriate patient management and avoiding extensive, unnecessary workups.