Vulvovaginal gingival syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare, non-malformative vulvovaginal disease characterized by a combination of erosive or desquamative lichen planus (LP) of vulval, vaginal and gingival mucosae, with a high propensity for scarring and stricture formation. Additional sites of involvement are frequently observed (in particular, tongue, buccal mucosae, skin and perianal LP). Patients may be asymptomatic or, more commonly, present with pain, burning, discomfort and bleeding, dyspareunia, and seropurulent vaginal discharge.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Vulvovaginal gingival syndrome | |
| fr | Syndrome vulvo-vaginal-gingival | |
| de | Vulvovagina-Gingiva-Syndrom | |
| es | Síndrome vulvovaginal-gingival | |
| it | Sindrome vulvovaginale-gengivale | |
| nl | Vulvovaginaal-gingivaal syndroom | |
| pt | Síndrome vulvo-vaginal - gengival | |
| pl | Zespół sromowo-waginalno-dziąsłowy | |
| cs | Vulvovaginogingivální syndrom | |
| uk | Вульвовагінальний ясенний синдром | |
| zh | 外阴阴道牙龈综合征 | |
| tr | Vulvovajinal dişeti sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L43.8 | NTBT | attributed | validated |
| ICD-11 | EA91.3 | NTBT | index | validated |
| MONDO | 0019370 | E | validated | |
| UMLS | C3873472 | 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 (2):
- ORPHA:254373Rare mucosal lichen planus
rare skin diseases - ORPHA:180205Rare non-malformative uterovaginal or vulvovaginal disease
rare gynecological and obstetric 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 | 380 cases | Worldwide | Case(s) | Validated | 20373919[PMID] | |
| Point prevalence | Unknown | — | 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.
HPO phenotypes
13 annotations · validated 2016-08-05| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0000230 | Gingivitis | Diagnostic criterion |
| Frequent (79-30%) | ||
| HP:0000055 | Abnormality of female external genitalia | |
| HP:0011118 | Abnormality of tumor necrosis factor secretion | |
| HP:0025092 | Epidermal acanthosis | |
| HP:0010783 | Erythema | |
| HP:0012537 | Food intolerance | |
| HP:0100725 | Lichenification | |
| HP:0000155 | Oral ulcer | |
| HP:0012531 | Pain | |
| HP:0001036 | Parakeratosis | |
| HP:0000989 | Pruritus | |
| HP:0200041 | Skin erosion | |
| Occasional (29-5%) | ||
| HP:0001807 | Ridged nail | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 21763554[PMID]_20373919[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/83453
- This record as JSON: /data/d/83453.json