Papular xanthoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly, Infancy
- Status
- active
Papular xanthoma is a form of non-Langerhans cell histiocytosis characterized by cutaneous presentation of solitary or disseminated yellow to orange-brown papular or papulonodular, noncoalescent, asymptomatic skin lesions located predominantly on the head, neck, trunk and extremities (rarely on oral mucosa), in the presence of normolipidemia. Microscopically, the lesions consist of monomorphous infiltrate of xanthomatized macrophages and numerous Touton giant cells, with scant or absent inflammatory infiltrate. It is usually not associated with systemic disease.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Papular xanthoma | |
| fr | Xanthome papuleux | |
| de | Xanthom, papuläres | |
| es | Xantoma papular | |
| it | Xantoma papuloso | |
| nl | Papulair xanthoom | |
| pt | Xantoma papular | |
| pl | Kępki żółte grudkowe | |
| cs | Papulární xantom | |
| uk | Папульозна ксантома | |
| zh | 丘疹性黄瘤 | |
| tr | Papüler ksantom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D76.3 | NTBT | attributed | validated |
| ICD-11 | EE81 | NTBT | index | validated |
| MONDO | 0015536 | E | validated | |
| UMLS | C4707373 | 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
4 annotations · validated 2017-04-26| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001013 | Eruptive xanthomas | |
| HP:0100727 | Histiocytosis | |
| HP:0200035 | Skin plaque | |
| Excluded (0%) | ||
| HP:0003077 | Hyperlipidemia | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 3888123[PMID]_23518438[PMID]_25258297[PMID]_15628330[PMID]_16638433[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/158008
- This record as JSON: /data/d/158008.json