Anti-p200 pemphigoid
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare, acquired, subepidermal autoimmune bullous disease characterized by polymorphic cutaneous lesions (blisters, urticarial lesions or scars/milia) associated with imunoglubulin G deposition in the basement membrane zone. Lesions are frequently localized on extremities, trunk, palmoplantar and cephalic areas as well as mucous membranes.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Anti-p200 pemphigoid | |
| fr | Pemphigoïde anti-p200 | |
| de | Anti-p200-Pemphigoid | |
| es | Penfigoide anti-p200 | |
| it | Pemfigoide anti-p200 | |
| nl | Anti-p200-pemfigoïd | |
| pt | Penfigoide anti-p200 | |
| pl | Pemfigoid anty-p200 | |
| cs | Anti-p200 pemfigoid | |
| uk | Анти-p200 пемфігоїд | |
| zh | 抗p-200类天疱疮 | |
| tr | Anti-p200 pemfigoid |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L12.8 | NTBT | attributed | validated |
| ICD-11 | EB41.Y | NTBT | index | validated |
| MONDO | 0018688 | E | validated | |
| UMLS | C5681099 | 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:79669Autoimmune bullous skin disease
rare skin 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 | 50 cases | Worldwide | Case(s) | Validated | 24767733[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_24767733[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/454710
- This record as JSON: /data/d/454710.json