Warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome
Synonyms: Disseminated warts-impaired cell-mediated immunity-primary lymphedema-anogenital dysplasia syndrome · WILD syndrome
- Type
- Disease
- Group
- Disorder
- Age of onset
- Infancy, Neonatal
- Status
- active
A rare primary lymphedema characterized by extensive, multisegmental lymphedema, associated with persistent, widespread infections with various genital high- and low-risk human papillomaviruses, resulting in multifocal anogenital dysplasia. Laboratory examination shows abnormalities in lymphocyte subsets, in particular CD4+ T-cells. Epidermal nevi and capillary malformations have also been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome | Disseminated warts-impaired cell-mediated immunity-primary lymphedema-anogenital dysplasia syndrome · WILD syndrome |
| fr | Syndrome de verrues multiples-déficit immunitaire-lymphoedème-dysplasies anogénitales | Syndrome WILD · Syndrome de verrues multiples-immunodéficience-lymphoedème-dysplasies anogénitales |
| de | Warzen-Immundefekt-Lymphödem-anogenitale Dysplasie-Syndrom | Disseminierte Warzen-gestörte zellvermittelte Immunität-primäres Lymphödem-anogenitale Dysplasie-Syndrom · WILD-Syndrom |
| es | Síndrome de verrugas-inmunodeficiencia-linfedema-displasia anogenital | Síndrome WILD · Síndrome de verrugas diseminadas-alteración de la inmunidad celular-linfedema primario-displasia anogenital |
| it | Sindrome ondilomi-immunodeficienza-linfedema-displasia ano-genitale | Sindrome WILD · Sindrome da condilomi disseminati, compromissione dell'immunità cellulo-mediata, linfedema primitivo e displasia ano-genitale |
| nl | Wratten - immuundeficiëntie - lymfoedeem - anogenitale dysplasie-syndroom | Gedissemineerde wratten - verstoorde celgemedieerde immuniteit - primair lymfoedeem - anogenitale dysplasie-syndroom · WILD-syndroom |
| pt | Síndrome de displasia anogenital-linfedema-imunodeficiência-Warts | |
| pl | Zespół brodawek, niedoboru odporności, obrzęku limfatycznego oraz dysplazji odbytu i narządów płciowych zewnętrznych | Zespół WILD |
| cs | Syndrom zahrnující bradavice, imunodeficienci, lymfedém a anogenitální dysplázii | = en |
| uk | Бородавки-імунодефіцит-лімфедема-аногенітальна дисплазія синдром | Синдром дисемінованих бородавок - порушення клітинного імунітету - первинної лімфедеми - аногенітальної дисплазії · Синдром WILD |
| zh | 疣-免疫缺陷-淋巴水肿-肛门生殖器发育不良综合征 | = en |
| tr | Siğiller-immün yetmezlik-lenfödem-anogenital displazi sendromu | Yaygın siğiller-bozulmuş hücre aracılı bağışıklık-primer lenfödem-anogenital displazi sendromu · WILD sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q82.0 | NTBT | attributed | validated |
| MONDO | 0035473 | E | validated | |
| UMLS | C5568569 | 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 (5):
- ORPHA:183494Genetic immune deficiency with skin involvement
rare genetic diseases - ORPHA:79391Immune deficiency with skin involvement
rare skin diseases - ORPHA:140162Inherited cancer-predisposing syndrome
rare genetic diseases; rare neoplastic diseases - ORPHA:331193Other immunodeficiency syndromes due to defects in innate immunity
rare genetic diseases; rare immunological diseases - ORPHA:568044Primary lymphedema with systemic or visceral involvement
rare developmental anomalies during embryogenesis; rare genetic diseases; rare skin diseases; rare circulatory system 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 | 2 cases | Worldwide | Case(s) | Validated | 18347293[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_18347293[PMID] |
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/568056
- This record as JSON: /data/d/568056.json