Kikuchi-Fujimoto disease
Synonyms: Histiocytic necrotizing lymphadenitis · Kikuchi disease
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Infancy
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Kikuchi-Fujimoto disease | Histiocytic necrotizing lymphadenitis · Kikuchi disease |
| fr | Maladie de Kikuchi-Fujimoto | Lymphadénite histiocytaire nécrosante · Maladie de Kikuchi |
| de | Kikuchi-Fujimoto-Krankheit | Kikuchi-Fujimoto-Lymphadenitis · Lymphadenitis, histiozytäre nekrotisierende · Kikuchi-Krankheit |
| es | Enfermedad de Kikuchi-Fujimoto | Enfermedad de Kikuchi |
| it | Malattia di Kikuchi-Fujimoto | Linfadenite istiocitica necrotizzante · Malattia di Kikuchi |
| nl | Ziekte van Kikuchi-Fujimoto | Hystiocytische necrotiserende lymfadenitis · Ziekte van Kikuchi |
| pt | Doença Kikuchi-Fujimoto | Doença de Kikuchi · Linfadenite necrotizante histiocítica |
| pl | Choroba Kikuchi i Fujimoto | Choroba Kikuchi · Histiocytarne martwicze zapalenie węzłów chłonnych |
| cs | Kikuchiho-Fujimotova choroba | Histiocytární nekrotizující lymfadenitida · Kikuchiho nemoc · Kikuchi-Fujimotova choroba |
| uk | Хвороба Кікучі-Фуджімото | Гістіоцитарний некротизуючий лімфаденіт · Хвороба Кікучі |
| zh | Kikuchi-Fujimoto病 | 组织细胞性坏死性淋巴结炎 |
| tr | Kikuchi-Fujimoto hastalığı | Kikuchi hastalığı · Histiositik nekrotizan lenfadenit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I88.1 | NTBT | attributed | validated |
| ICD-11 | 4B2Y | NTBT | index | validated |
| MONDO | 0018864 | E | validated | |
| MeSH | D020042 | E | validated | |
| UMLS | C0398367 | E | validated | |
| GARD | 6834 | E | validated | |
| MedDRA | 10069070 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:182222Rare systemic disease
rare systemic and rheumatological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_25500707[PMID] |
| Cases/families | 1052 cases | Worldwide | Case(s) | Validated | 25500707[PMID] | |
| Annual incidence | 1-9 / 1 000 000 | 0.278 / 100 000 | Martinique | Value and class | Validated | 27015112[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
50 annotations · validated 2016-08-04| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0025289 | Cervical lymphadenopathy | |
| Frequent (79-30%) | ||
| HP:0002733 | Abnormality of the lymph nodes | Diagnostic criterion |
| HP:0001596 | Alopecia | |
| HP:0002039 | Anorexia | |
| HP:0025143 | Chills | |
| HP:0000992 | Cutaneous photosensitivity | |
| HP:0010783 | Erythema | |
| HP:0012378 | Fatigue | |
| HP:0025435 | Increased circulating lactate dehydrogenase concentration | |
| HP:0001882 | Leukopenia | |
| HP:0011134 | Low-grade fever | |
| HP:0002716 | Lymphadenopathy | Diagnostic criterion |
| HP:0025300 | Malar rash | |
| HP:0030166 | Night sweats | |
| HP:0000155 | Oral ulcer | |
| HP:0100540 | Palpebral edema | |
| HP:0000989 | Pruritus | |
| HP:0200036 | Skin nodule | |
| HP:0000988 | Skin rash | |
| HP:0002633 | Vasculitis | |
| HP:0200029 | Vasculitis in the skin | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0006530 | Abnormal pulmonary interstitial morphology | |
| HP:0011024 | Abnormality of the gastrointestinal tract | |
| HP:0000464 | Abnormality of the neck | Diagnostic criterion |
| HP:0001903 | Anemia | |
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0002829 | Arthralgia | |
| HP:0001875 | Decreased total neutrophil count | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0011801 | Enlargement of parotid gland | |
| HP:0025475 | Erythematous macule | |
| HP:0100827 | Lymphocytosis | |
| HP:0012733 | Macule | |
| HP:0001287 | Meningitis | |
| HP:0003326 | Myalgia | |
| HP:0200034 | Papule | |
| HP:0200041 | Skin erosion | |
| HP:0200035 | Skin plaque | |
| HP:0001744 | Splenomegaly | |
| HP:0001873 | Thrombocytopenia | |
| Very rare (<4-1%) | ||
| HP:0001251 | Ataxia | |
| HP:0008940 | Generalized lymphadenopathy | |
| HP:0002240 | Hepatomegaly | |
| HP:0012819 | Myocarditis | |
| HP:0002202 | Pleural effusion | |
| HP:0200039 | Pustule | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 26627522[PMID]_27207311[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/50918
- This record as JSON: /data/d/50918.json