KIKUCHI-FUJIMOTO disease (histiocytic necrotizing lymphadenitis)
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).
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
- 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
- Weight Loss (10%)
- 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
- 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
- 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)
- Kawasaki vasculitis in children
- Hodgkin's lymphoma (Diagnosis by lymph node biopsy)
Treatment / follow-up
Check to exclude SLE development after a few years.
Self-limiting disease. Spontaneous decline after 1 - 4 months.
- Alnes M, 2020
- Mauleom C, 2012 (Mimicking cutaneous SLE)
- Wikipedia (English)
- Grans Compendium in Rheumatology