ORPHA:254478
Lichen planus pemphigoides
Synonyms: LP pemphigoides
- Type
- Disease
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
Lichen planus (LP) pemphigoides is a rare cross-over syndrome between lichen planus and bullous pemphigoid.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Lichen planus pemphigoides | LP pemphigoides |
| fr | Lichen plan pemphigoïde | LP pemphigoïde |
| de | Lichen planus pemphigoides | LP, pemphigoider |
| es | Liquen plano penfigoide | |
| it | Lichen planus pemfigoide | LP pemfigoide |
| nl | Lichen planus pemphigoides | = en |
| pt | Líquen plano penfigoide | LP penfigoide |
| pl | Liszaj płaski pemfingoidalny | LP pemfingoidalny |
| cs | Pemfigoidní lichen planus | Pemfigoidní LP |
| uk | Плескатий лишай пемфігоїдний | = en |
| zh | 天疱疮样扁平苔藓 | 天疱疮样LP |
| tr | Liken planus pemphigoides | LP pemfigoidleri |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L43.1 | NTBT | attributed | validated |
| ICD-11 | EB41.Y | NTBT | index | validated |
| MONDO | 0016775 | E | validated | |
| UMLS | C0406369 | E | validated | |
| GARD | 12677 | E | validated | |
| MedDRA | 10087934 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:254370Rare cutaneous lichen planus
rare skin diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 100 cases | Worldwide | Case(s) | Validated | 23237497[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | 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.
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HPO phenotypes
10 annotations · validated 2016-10-21| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000962 | Hyperkeratosis | |
| HP:0007535 | Hypopigmented streaks | |
| HP:0100725 | Lichenification | |
| HP:0200037 | Skin vesicle | |
| Frequent (79-30%) | ||
| HP:0011830 | Abnormal oral mucosa morphology | |
| HP:0000989 | Pruritus | |
| Very rare (<4-1%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0001597 | Abnormal nail morphology | |
| HP:0000498 | Blepharitis | |
| HP:0000509 | Conjunctivitis | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 23331194[PMID]_23626545[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/254478
- This record as JSON: /data/d/254478.json