ORPHA:63455
Paraneoplastic pemphigus
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare form of autoimmune bullous skin disease characterized by polyformative skin lesions, typically beginning on the oral mucus membranes, and generally associated with lymphoma or chronic lymphoid leukemia.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Paraneoplastic pemphigus | |
| fr | Pemphigus paranéoplasique | |
| de | Pemphigus, paraneoplastischer | |
| es | Penfigoide paraneoplásico | |
| it | Pemfigo paraneoplastico | |
| nl | Paraneoplastische pemfigus | Pemphigus paraneoplastica |
| pt | Pênfigo paraneoplásico | |
| pl | Pęcherzyca paraneoplastyczna | |
| cs | Paraneoplastický pemfigus | |
| uk | Паранеопластичний пемфігус | |
| zh | 副肿瘤性天疱疮 | |
| tr | Paraneoplastik pemfigus |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L10.8 | NTBT | attributed | validated |
| ICD-11 | EB40.2 | E | specific | validated |
| MONDO | 0018974 | E | validated | |
| UMLS | C1112570 | E | validated | |
| MedDRA | 10057056 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:79669Autoimmune bullous skin disease
rare skin diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | 31536300[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.
6
HPO phenotypes
7 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0200097 | Oral mucosal blisters | |
| HP:0000155 | Oral ulcer | |
| HP:0200041 | Skin erosion | |
| Frequent (79-30%) | ||
| HP:0012191 | B-cell lymphoma | |
| HP:0100242 | Sarcoma | |
| HP:0100522 | Thymoma | |
Run the differential on the 4 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/63455
- This record as JSON: /data/d/63455.json