Congenital erosive and vesicular dermatosis
Synonyms: CEVD · Congenital erosive and vesicular dermatosis with reticulated supple scarring
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Infancy, Neonatal
- Status
- active
A rare, idiopathic skin disease characterized by widespread, congenital, superficial erosions and vesicles (often involving more than 75% of the body) which heal leaving scars with a supple, symmetrical, reticulated pattern, frequently resulting in cicatricial alopecia and hyperthermia and/or hypohydrosis. Nail anomalies, neurodevelopmental and ophtalmologic abnormalities, tongue atrophy and preterm birth, with or without history of choriomnionitis, are commonly associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital erosive and vesicular dermatosis | CEVD · Congenital erosive and vesicular dermatosis with reticulated supple scarring |
| fr | Dermatose érosive et vésiculaire congénitale | Dermatose érosive et vésiculeuse congénitale avec cicatrices réticulées |
| de | Dermatose, erosive und vesikuläre, kongenital | CEVD · Dermatose, erosive und vesikuläre, kongenitale, mit retikulärer weicher Vernarbung |
| es | Dermatosis erosiva y vesicular congénita | CEVD · Dermatosis vesicular y erosiva congénita con cicatrización flexible reticulada |
| it | Dermatosi vescicolare ed erosiva congenita | |
| nl | Congenitale erosieve en vesiculaire dermatose | CEVD · Congenitale erosieve en vesiculaire dermatose met reticulaire soepele littekens |
| pt | Dermatose erosiva e vesicular, congénita | |
| pl | Wrodzona nadżerkowa i pęcherzykowa choroba skóry | = en |
| cs | Vrozená erozivní a vezikulární dermatóza | = en |
| uk | Вроджені ерозивні та везикулярні дерматози | CEVD · Вроджений ерозивний та везикулярний дерматоз з сітчастим еластичним рубцюванням |
| zh | 先天性糜烂和水疱性皮肤病 | 先天性糜烂和水疱性皮肤病伴网状柔软瘢痕 |
| tr | Doğumsal aşındırıcı ve veziküler dermatoz | CEVD · Retiküle esnek yara izi ile seyreden doğumsal eroziv ve veziküler dermatoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q82.8 | NTBT | attributed | validated |
| ICD-11 | KC31 | E | specific | validated |
| MONDO | 0016503 | E | validated | |
| UMLS | C4751164 | 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:79359Other epidermal disorder
rare skin 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 | 31 cases | Worldwide | Case(s) | Validated | 28247410[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_28247410[PMID] |
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/231573
- This record as JSON: /data/d/231573.json