Linear focal elastosis
Synonyms: Elastotic striae · Linear focal dermal elastosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Linear focal elastosis is a rare, acquired, dermis elastic tissue disorder characterized by asymptomatic, palpable, hypertrophic or atrophic, yellowish or red, indurated, horizontal, striae-like linear plaques distributed symmetrically across the mid and lower back. No systemic involvement has been described. Skin biopsy reveals a focal increase in abnormal elastic tissue with abundant, wavy, fragmented and aggregated, basophilic elastic fibers in the reticular dermis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Linear focal elastosis | Elastotic striae · Linear focal dermal elastosis |
| fr | Elastose focale linéaire | |
| de | Elastose, fokale lineare | Elastotische Striae · Lineare fokale dermale Elastose |
| es | Elastosis focal lineal | Elastosis dérmica focal lineal · Estrías elastóticas |
| it | Elastosi focale lineare | Strie elastotiche |
| nl | Lineaire focale elastose | Elastotische striae · Lineaire focale dermale elastose |
| pt | Elastose dérmica focal linear | |
| pl | Linijna elastoza ogniskowa skóry | Rozstępy elastotyczne |
| cs | Lineární fokální dermální elastóza | Elastotické strie |
| uk | Лінійний вогнищевий еластоз | Еластичні смуги · Лінійний вогнищевий дермальний еластоз |
| zh | 线性局灶性弹性组织变性 | 线性局灶性真皮弹性组织变性 |
| tr | Lineer fokal elastoz | Elastotik çizgiler · Doğrusal fokal dermal elastoz |
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 | attributed | validated |
| MONDO | 0016438 | E | validated | |
| UMLS | C4751166 | 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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 30 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/228236
- This record as JSON: /data/d/228236.json