KIKUCHI-FUJIMOTO disease (histiocytic necrotizing lymphadenitis)

Definition

Kikutchi-Fujimoto Disease (KFS) is a rare, benign, necrotizing lymphadenopathy (lymph node disease), most often on the neck of young women. Histology (tissue examination in biopsy) separates from cancer: histiocytic necrotizing. Unknown cause. The disease is in some cases associated with Systemic lupus erythematosus (SLE).

Occurrence

Most are young women, the disease can also affect men. Typically under 40 years of age at onset, but reported between 6 - 80 years of age. The average age at onset is 30 years.

• All ethnic groups can get the disease, but most often in Asia (reference: Mrowka-Kata K, 2013).
• The disease is sometimes associated with Systemic connective tissue disease, especially Systemic lupus (reference: Baena's DF, 2016).
• Kikutchi-Fujimoto disease is defined as one rare disease

Symptoms

• Swollen lymph nodes on the neck (100%, 1 - 2 cm in diameter, up to 7 cm described)
• Fever (30-50%, 1-4 weeks) at debut
• Night sweats
• Dizziness
• Headache
• Vomit
• Diarrhea
• Weight Loss (10%)
• Rash
  • Eczema (10%, as in red dogs (Rubella-like)
• Arthritis (5-7%)
• Large liver and spleen (hepatosplenomegaly) (3%)
• Bone marrow: Increased number of macrophages, no atypical cells
• SLE Association occurs, but ANA test is usually normal

Medical examination

Blood tests

• Many have normal samples. Low white blood cell count (Leukopenia, in 20-32%, Blood drop reaction (SR) over 60mm in 40 - 70%, anemia in 23%)

Tissue test (biopsy) is crucial for the diagnosis

Diagnosis

Lymph node biopsy (different from lymphoma: Hodgkins, non-Hodgkins).

• Thin needle biopsy can be used. immunohistochemistry
  • CD68 + T lymphocytes are detected
  • Absence of neutrophils in the "necrotizing phase" differs from SLE

CT examination of swollen lymph nodes shows perinodular infiltration (81%), homogeneous contrast recording (83%)

Incorrect diagnosis? (Similar diseases / differential diagnoses)

• SLE (ANA is expected to be positive in active SLE disease with swollen lymph nodes)
• Infection
  • EBV (Mononucleosis)
  • Cytomegaly virus
  • HIV
  • Toxoplasmosis
  • Cat scratch disease
  • Tuberculosis (Tbc)
• Kawasaki vasculitis in children
• Hodgkin's lymphoma (Diagnosis by lymph node biopsy)

Treatment / follow-up

No specific therapy. For severe symptoms can corticosteroids (Prednisone) be useful.

Check to exclude SLE development after a few years.

Prognosis

Self-limiting disease. Spontaneous decline after 1 - 4 months.

Literature

• Alnes M, 2020
• Mauleom C, 2012 (Mimicking cutaneous SLE)
• Wikipedia (English)
• Grans Compendium in Rheumatology

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