Oral submucous fibrosis
Synonyms: OSMF
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Oral submucous fibrosis (OSMF) is a chronic, progressive disease that alters the fibroelasticity of the oral submucosa, prevalent in India and Southeast Asia but rare elsewhere, and characterized by burning and pain in the oral cavity, loss of gustatory sensation, the presence of blanched fibrous bands and stiffening of the oral mucosa and oro-pharynx (leading to trismus and a progressive reduction in mouth opening) and an increased risk of developing oral squamous cell cancer (3-19%). It is usually associated with the chewing of the areca nut (an ingredient in betel quid) but the exact etiology is unknown and there is currently no effective treatment.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Oral submucous fibrosis | OSMF |
| fr | Fibrose buccale sous-muqueuse | = en |
| de | Orale submuköse Fibrose | = en |
| es | Fibrosis submucosa oral | = en |
| it | Fibrosi orale sottomucosa | = en |
| nl | Orale submuceuze fibrose | = en |
| pt | Fibrose oral submucosa | |
| pl | Zwłóknienie podśluzówkowe jamy ustnej | = en |
| cs | Orální submukózní fibróza | = en |
| uk | Підслизовий фіброз ротової порожнини | = en |
| zh | 口腔粘膜下纤维性变 | = en |
| tr | Oral submukoz fibroz | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K13.5 | E | specific | validated |
| ICD-11 | DA02.2 | E | specific | validated |
| MONDO | 0018166 | E | validated | |
| MeSH | D009914 | E | validated | |
| UMLS | C0029172 | E | validated | |
| GARD | 7264 | E | validated | |
| MedDRA | 10031023 | 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:68329Rare maxillo-facial surgical disease
rare surgical maxillo-facial diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | 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
7 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000163 | Abnormal oral cavity morphology | |
| HP:0000600 | Abnormality of the pharynx | |
| HP:0100825 | Cheilitis | |
| HP:0000160 | Narrow mouth | |
| Frequent (79-30%) | ||
| HP:0001371 | Flexion contracture | |
| HP:0000211 | Trismus | |
| Occasional (29-5%) | ||
| HP:0012182 | Oropharyngeal squamous cell carcinoma | |
Run the differential on the 4 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/357154
- This record as JSON: /data/d/357154.json