Necrobiotic xanthogranuloma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
Necrobiotic xanthogranuloma is a rare, chronic and progressive, non-Langerhans cell histiocytosis disease typically characterized by multiple, indurated, asymptomatic to pruritic, yellow-orange plaques or nodules that tend to ulcerate and are usually located in the periorbital area, trunk and/or extremities. Strong association with paraproteinemia and/or malignant lymphoproliferative disease has been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Necrobiotic xanthogranuloma | |
| fr | Xanthogranulome nécrobiotique | |
| de | Xanthogranulom, nekrobiotisches | |
| es | Xantogranuloma necrobiótico | |
| it | Xantogranuloma necrobiotico | |
| nl | Necrobiotisch xanthogranuloom | |
| pt | Xantogranuloma necrobiótico | |
| pl | Żółtakoziarniniakowatość z nekrobiozą | |
| cs | Nekrobiotický xantogranulom | |
| uk | Некробіотична ксантогранульома | |
| zh | 渐进性坏死性黄肉芽肿 | |
| tr | Nekrobiyotik ksantogranülom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D76.3 | NTBT | attributed | validated |
| ICD-11 | EE8Y | NTBT | index | validated |
| MONDO | 0015537 | E | validated | |
| MeSH | D058252 | E | validated | |
| UMLS | C1275339 | E | validated | |
| GARD | 10951 | 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:157987Non-Langerhans cell histiocytosis
rare skin diseases; rare systemic and rheumatological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/158011
- This record as JSON: /data/d/158011.json