Kimura disease
Synonyms: Eosinophilic lymphogranuloma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adult
- Status
- active
A rare systemic disease characterized by chronic inflammation of unknown etiology and occurring predominantly in males in the second to third decades of life, mainly in Southeast Asian countries. Patients present with painless soft tissue swelling most commonly involving the head and neck region, usually with salivary gland involvement (parotid and submandibular glands) and regional lymphadenopathy. Frequent laboratory findings are peripheral eosinophilia and raised serum IgE. The diagnosis is confirmed histopathologically by the presence of well-formed lymphoid follicles with eosinophilic infiltration and vascular proliferation in the biopsy.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Kimura disease | Eosinophilic lymphogranuloma |
| fr | Maladie de Kimura | Lymphogranulome éosinophile |
| de | Kimura-Krankheit | Lymphogranulom, eosinophiles |
| es | Enfermedad de Kimura | |
| it | Malattia di Kimura | Linfogranuloma eosinofilo |
| nl | Ziekte van Kimura | Eosinofiel lymfogranuloom |
| pt | Doença Kimura | Linfogranuloma eosinofílico |
| pl | Choroba Kimura | Eozynofilny ziarniniak limfoidalny |
| cs | Kimurova choroba | Eozinofilní lymfogranulom |
| uk | Хвороба Кімура | Еозинофільна лімфогранулема |
| zh | 木村病 | 嗜酸性淋巴肉芽肿 |
| tr | Kimura hastalığı | Eozinofilik lenfogranülom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D21.9 | NTBT | index | validated |
| ICD-11 | 4B2Y | NTBT | index | validated |
| MONDO | 0018830 | E | validated | |
| MeSH | D000082242 | E | validated | |
| UMLS | C0033838 | E | validated | |
| GARD | 6835 | E | validated | |
| MedDRA | 10048640 | 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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 300 cases | Worldwide | Case(s) | Validated | 18808562[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET |
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
5 annotations · validated 2016-07-07| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002729 | Follicular hyperplasia | |
| HP:0003212 | Increased circulating IgE concentration | |
| HP:0001880 | Increased total eosinophil count | |
| Frequent (79-30%) | ||
| HP:0002716 | Lymphadenopathy | |
| Occasional (29-5%) | ||
| HP:0010286 | Abnormal salivary gland morphology | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 15087670[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/482
- This record as JSON: /data/d/482.json