Kikuchi-Fujimoto disease

Kikuchi-Fujimoto disease (KFD) (also known as subacute necrotising lymphadenitis or subacute necrotising histiocytosis) is an idiopathic disease characterised usually by cervical lymph node enlargement (80%).

It typically affects young women.

It usually presents clinically with cervical lymphadenopathy that is sometimes tender. Accompanying systemic symptoms include low-grade fever, night sweats, malaise and joint or abdominal pains resembling a viral illness 3,4.

The cause is unknown and debated, with both viral infection and autoimmune mediated mechanisms proposed in the literature.

It can histologically be mistaken for systemic lupus erythematosus (SLE). In some cases, patients go on to develop SLE, raising the possibility that KFD is a forme fruste of SLE.

Location

Typically (~80%) a single nodal group is involved, most frequently (70%) the posterior triangle. The nodes are soft and sometimes tender.

Imaging findings are non-specific and demonstrate primarily cervical nodal enlargement. The modality used depends on the suspected differential diagnoses at the time of presentation.

A review of CT neck findings in patients with Kikuchi disease to characterise nodal findings showed 6:

  • homogenous nodal enlargement (83%)
  • perinodal infiltration (80%)
  • central nodal necrotic change (16%)

Post-contrast enhancement has been reported.

It usually runs a benign course that is self limiting with most lymph nodes returning to normal size within the first six months of diagnosis.

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Article Information

rID: 1554
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Kikuchi disease
  • Subacute necrotising lymphadenitis
  • Subacute necrotizing lymphadenitis
  • subacute necrotising histiocytosis
  • subacute necrotizing histiocytosis
  • Kikuchi-Fujimoto disease (KFD)
  • Histiocytic necrotizing lymphadenitis
  • Histiocytic necrotising lymphadenitis

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Cases and Figures

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    Case 1: cervical lymphadenopathy - ultrasound
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