Localized pagetoid reticulosis
Synonyms: Pagetoid reticulosis, Woringer-Kolopp type
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare variant of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, characterized by the presence of localized patches or plaques with epidermal hyperplasia and intraepidermal proliferation of neoplastic T-cells, usually involving one extremity.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Localized pagetoid reticulosis | Pagetoid reticulosis, Woringer-Kolopp type |
| fr | Réticulose pagétoïde localisée | Réticulose pagétoïde type Woringer-Kolopp |
| de | Retikulose, lokalisierte pagetoide | Pagetoide Retikulose Typ Woringer-Kolopp |
| es | Reticulosis pagetoide localizada | Reticulosis pagetoide tipo Woringer-Kolopp |
| it | Reticulosi localizzata pagetoide | |
| nl | Gelokaliseerde pagetoïde reticulose | Pagetoïde reticulose, Woringer-Kolopp-type |
| pt | Reticulose pagetóide localizada | |
| pl | Zlokalizowana siatkowica pagetoidalna | Siatkowica pagetoidalna, typu Woringera i Koloppa |
| cs | Lokalizovaná pagetoidní retikulóza | Pagetoidní retikulóza, Woringerův-Koloppův typ · Pagetoidní retikulóza, Woringer-Koloppův typ |
| uk | Локалізований пагетоїдний ретикулоз | Пагетоїдний ретикулоз, тип Ворінгера-Колоппа |
| zh | 局限性畸形性骨炎网状细胞增多症 | Woringer-Kolopp型变形性骨炎网状细胞增多症 |
| tr | Lokalize pagetoid retiküloz | Pagetoid retiküloz, Woringer-Kolopp tipi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C84.0 | NTBT | attributed | validated |
| ICD-11 | 2B01 | NTBT | attributed | validated |
| MONDO | 0015809 | E | validated | |
| MeSH | D056267 | E | validated | |
| UMLS | C1276140 | 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:178566Mycosis fungoides and variants
rare skin diseases; rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet 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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/178517
- This record as JSON: /data/d/178517.json