Ocular cicatricial pemphigoid
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare inflammatory eye disease characterized by sub-epithelial blistering manifesting with bilateral, asymmetrical, chronic or recurrent conjunctivitis and aberrant tissue regeneration leading to progressive conjunctival fibrosis, secondary corneal vascularization and, in some cases, blindness. Patients typically present with conjunctival redness, increased lacrimation, burning and/or foreign body sensation, edema, limbitis and/or varying degrees of ocular pain. Ankyloblepharon may be observed in end stages of the disease.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Ocular cicatricial pemphigoid | |
| fr | Pemphigoïde oculaire cicatricielle | |
| de | Pemphigoid, okuläres vernarbendes | |
| es | Penfigoide ocular cicatricial | |
| it | Pemfigoide cicatriziale oculare | |
| nl | Oculair cicatricieel pemfigoïd | |
| pt | Penfigóide ocular cicatricial | |
| pl | Pemfigoid bliznowaciejący oczny | |
| cs | Oční pemfigoid | |
| uk | Очний рубцевий пемфігоїд | |
| zh | 眼部瘢痕性类天疱疮 | |
| tr | Oküler sikatrisyel pemfigoid |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H13.3* | NTBT | inclusion | validated |
| ICD-10 | L12.1 | E | specific | validated |
| ICD-11 | 9A62 | E | specific | validated |
| MONDO | 0008109 | E | validated | |
| MONDO | 8109 | E | validated | |
| UMLS | C1282359 | E | validated | |
| GARD | 8759 | E | validated | |
| MedDRA | 10067776 | 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:98610Rare disorder with conjunctival involvement as a major feature
rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99922
- This record as JSON: /data/d/99922.json