Juvenile xanthogranuloma
Synonyms: JXG
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Childhood, Infancy, Neonatal
- Status
- active
Juvenile xanthogranuloma is the most common type of non-Langerhans cell histiocytosis characterized by the occurrence of one or more reddish or yellowish self-limiting and benign papules or nodules of several millimeters in diameter, usually appearing on the head and neck (but sometimes on the extremities and trunk) during the first year of life (or rarely in adulthood) and usually regressing spontaneously. Extracutaneous involvement has also been reported, involving most commonly the eye (uveal tract) but with other locations including the central nervous system, lung, liver, bones and endocrine glands, and may be associated with considerable morbidity.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Juvenile xanthogranuloma | JXG |
| fr | Xanthogranulome juvénile | = en |
| de | Xanthogranulom, juveniles | = en |
| es | Xantogranuloma juvenil | |
| it | Xantogranuloma giovanile | = en |
| nl | Juveniel xanthogranuloom | = en |
| pt | Xantogranuloma juvenil | = en |
| pl | Żółtakoziarniniakowatość młodzieńcza | Żółtakoziarniniak młodzieńczy |
| cs | Juvenilní xantogranulom | = en |
| uk | Ювенільна ксантогранульома | = en |
| zh | 幼年性黄色肉芽肿 | |
| tr | Juvenil ksantogranülom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D76.3 | NTBT | attributed | validated |
| ICD-11 | 2B31.0 | E | specific | validated |
| MONDO | 0015534 | E | validated | |
| MeSH | D014972 | E | validated | |
| UMLS | C0043324 | E | validated | |
| MedDRA | 10089466 | 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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.005 / 100 000 | Germany | Value and class | Validated | 22969141[PMID] |
| Point prevalence | <1 / 1 000 000 | 0.0225 / 100 000 | Germany | Value and class | Validated | 22969141[PMID]_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
12 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Occasional (29-5%) | ||
| HP:0011830 | Abnormal oral mucosa morphology | |
| HP:0002086 | Abnormality of the respiratory system | |
| HP:0200064 | Asymmetry of iris pigmentation | |
| HP:0000498 | Blepharitis | |
| HP:0000501 | Glaucoma | |
| HP:0011886 | Hyphema | |
| HP:0001101 | Iritis | |
| HP:0007565 | Multiple cafe-au-lait spots | |
| HP:0005547 | Myeloproliferative disorder | |
| HP:0000520 | Proptosis | |
| HP:0000554 | Uveitis | |
| HP:0000572 | Visual loss | |
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/158000
- This record as JSON: /data/d/158000.json