Mid-dermal elastolysis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare, acquired, dermis elastic tissue disease characterized by asymptomatic, well-demarcated, symmetric patches and/or plaques of finely wrinkled skin arranged parallel to skin cleavage lines (type I), associated with perifollicular papular protrusions (type II) or with persistent reticular erythema (type III), occurring predominantly on the shoulders, trunk, back, and proximal extremities, associating, on histopathology, a selective loss of elastic tissue in the midreticular dermis. Erythema and/or urticaria may or may not precede wrinkly lesions.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Mid-dermal elastolysis | |
| fr | Elastolyse du derme moyen | |
| de | Mid-dermale Elastolyse | |
| es | Elastolisis de la dermis media | |
| it | Elastolisi del derma medio | |
| nl | Mid-dermale elastolyse | |
| pt | Elastólise da derme média | |
| pl | Elastoliza śródskórna | |
| cs | Mid-dermální elastolýza | |
| uk | Серньодермальний еластоз | |
| zh | 皮肤中度弹性组织离解 | |
| tr | Orta dermal elastoliz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L98.8 | NTBT | attributed | validated |
| ICD-11 | EE41.Y | NTBT | index | validated |
| MONDO | 0016449 | E | validated | |
| UMLS | C4728147 | E | validated | |
| MedDRA | 10081688 | 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):
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/228299
- This record as JSON: /data/d/228299.json