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Start free with EleplanKikuchi-Fujimoto disease
ORPHA:50918Disease
Also called Histiocytic necrotizing lymphadenitis · Kikuchi disease
What it is
A rare systemic disease characterized by subacute, necrotizing, regional lymphadenopathy with tenderness, usually accompanied with mild fever, night sweats, myalgia, leukopenia and anemia. Less frequent symptoms include weight loss, nausea, vomiting and sore throat. It is a self-limiting benign disease that usually recedes within six months, even without drug treatment.
Key facts
- Prevalence
- 1-9 / 1 000 000 (annual incidence, Martinique)
- Age of onset
- Adolescent, Adult, Childhood, Infancy
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
21- Abnormality of the lymph nodesDiagnostic criterion
- Alopecia
- Anorexia
- Chills
- Cutaneous photosensitivity
- Erythema
- Fatigue
- Increased circulating lactate dehydrogenase concentration
- Leukopenia
- Low-grade fever
- LymphadenopathyDiagnostic criterion
- Malar rash
- Night sweats
- Oral ulcer
- Palpebral edema
- Pruritus
- Skin nodule
- Skin rash
- Vasculitis
- Vasculitis in the skin
- Weight loss
Sometimes5–29%
22- Abnormal blistering of the skin
- Abnormality of the gastrointestinal tract
- Abnormality of the neckDiagnostic criterion
- Abnormal pulmonary interstitial morphology
- Anemia
- Antinuclear antibody positivity
- Arthralgia
- Decreased total neutrophil count
and 14 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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