Kikutchi-Fujimoto disease with swollen lymph node on the neck. Khadanga S, 2014. Openi, CC BY NC SA 3.0
Contents
KIKUCHI-FUJIMOTO disease (histocytic 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 the 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 tests. Low white blood cell count (Leukopenia, in 20-32%, Blood Reduction Response (SR) over 60mm in 40 - 70%, anemia in 23%)
Tissue test (biopsy) is crucial for the diagnosis
Diagnosis
Lymph node biopsy (distinguish from lymphoma: Hodgkin, non-Hodgkin).
- 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
- 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 illness. Spontaneous decline after 1 - 4 months.
