ORPHA:692256
Isolated anogenital granulomatosis
Synonyms: AGG
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare urogenital disease characterized by the presence of granulomas in the genital or anoperineal area presenting with painless swelling, inflammation, ulcers and lymphoedema. It is usually associated with a history of recurrent perianal or genital lesions.
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Names in Orphadata languages
9 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Isolated anogenital granulomatosis | AGG |
| fr | Granulomatose anogénitale isolée | GAG |
| de | Isolierte anogenitale Granulomatose | = en |
| es | Granulomatosis anogenital aislada | = en |
| it | Granulomatosi anogenitale isolata | = en |
| nl | Geïsoleerde anogenitale granulomatose | = en |
| pt | Granulomatose anogenital isolada | = en |
| pl | Izolowana ziarniniakowatość odbytu i narządów płciowych | = en |
| cs | Izolovaná anogenitální granulomatóza | = en |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L29.3 | NTBT | attributed | validated |
| ICD-11 | EC90.5 | NTBT | attributed | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- ORPHA:79381Other dermis disorder
rare skin diseases - ORPHA:180205Rare non-malformative uterovaginal or vulvovaginal disease
rare gynecological and obstetric diseases - ORPHA:101433Rare urogenital disease
rare urogenital diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | 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.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/692256
- This record as JSON: /data/d/692256.json