Erythrokeratoderma variabilis progressiva
- Type
- Clinical group
- Group
- Group of disorders
- Age of onset
- Childhood, Infancy, Neonatal
- Status
- active
Erythrokeratoderma variabilis progressiva (EKVP) is a type of erythrokeratoderma characterized by the association of hyperkeratosis and erythema in persistent, although sometimes variable, circumscribed lesions. Progressive symmetric erythrokeratoderma (PSEK) and erythrokeratoderma variabilis (EKV) are probably no longer two distinctive diseases but rather the two clinical manifestations of a same disease, now known as EKVP.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Erythrokeratoderma variabilis progressiva | |
| fr | Erythrokeratodermie variable progressive | |
| de | Erythrokeratodermie, progressive variable | |
| es | Eritroqueratodermia variable progresiva | |
| it | Eritrocheratoderma variabilis progressiva | |
| nl | Erythrokeratodermie variabilis progressiva | |
| pt | Eritoqueratodermia variabilis progressiva | |
| pl | Erytrokeratodermia zmienna postępująca | |
| cs | Erythrokeratoderma variabilis progressiva | |
| uk | Еритрокератодермія варіабельна прогресуюча | |
| zh | 变异性红皮角化病 | |
| tr | Eritrokeratoderma variabilis progresiva |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0017681 | E | validated | |
| MONDO | 17851 | E | validated | |
| UMLS | C5681068 | E | validated | |
| GARD | 10923 | 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:79355Erythrokeratoderma
rare skin diseases - ORPHA:183438Genetic erythrokeratoderma
rare genetic diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | <1 / 1 000 000 | 0.046 / 100 000 | France | Value and class | Validated | 24393603[PMID]_[EXPERT] |
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/308166
- This record as JSON: /data/d/308166.json