Lymphomatoid papulosis
Synonyms: LyP
- Type
- Disease
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
Lymphomatoid papulosis (LyP) is a rare cutaneous condition characterized by chronic, recurrent, and self-regressing papulonodular skin eruptions. It belongs to the spectrum of primary cutaneous CD30+ lymphoproliferative disorders, along with primary cutaneous anaplastic large cell lymphoma (primary C-ALCL) with which it shares overlapping clinical and histopathologic features.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Lymphomatoid papulosis | LyP |
| fr | Papulose lymphomatoïde | |
| de | Papulose, lymphomatoide | = en |
| es | Papulosis linfomatoide | = en |
| it | Papulosi linfomatoide | = en |
| nl | Lymfomatoïde papulose | = en |
| pt | Papulose linfomatóide | |
| pl | Grudkowatość limfoidalna | = en |
| cs | Lymfomatoidní papulóza | = en |
| uk | Лімфоматоїдний папулез | = en |
| zh | 淋巴瘤样丘疹病 | = en |
| tr | Lenfomatoid papüloz | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C86.6 | NTBT | inclusion | validated |
| ICD-11 | 2B03.1 | E | specific | validated |
| MONDO | 0020326 | E | validated | |
| MeSH | D017731 | E | validated | |
| UMLS | C0206182 | E | validated | |
| GARD | 6944 | E | validated | |
| MedDRA | 10056670 | 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:541Primary cutaneous CD30+ T-cell lymphoproliferative disease
rare skin diseases; rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| NPM1 | Part of a fusion gene in | Assessed | HGNC:7910 | ENSG00000181163 | 164040 | 5q35.1 | nucleophosmin 1 | 25349176[PMID] |
| TYK2 | Part of a fusion gene in | Assessed | HGNC:12440 | ENSG00000105397 | 176941 | 19p13.2 | tyrosine kinase 2 | 25349176[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Europe | 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/98842
- This record as JSON: /data/d/98842.json