Late-onset focal dermal elastosis
Synonyms: PXE-like late-onset focal dermal elastosis · Pseudoxanthoma-like late-onset focal dermal elastosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Late-onset focal dermal elastosis is a rare, acquired, dermis elastic tissue disorder characterized by a pseudoxanthoma elasticum-like papular eruption consisting of multiple, slowly progressive, asymptomatic, 2-5 mm, white to yellowish, non-follicular papules (that tend to form cobblestone plaques) predominantly distributed over the neck, axillae and flexural areas, with no systemic involvement. Skin biopsy reveals a focal increase of normal-appearing elastic tissue in the reticular dermis with no calcium deposits.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Late-onset focal dermal elastosis | PXE-like late-onset focal dermal elastosis · Pseudoxanthoma-like late-onset focal dermal elastosis |
| fr | Elastose dermique focale tardive | Elastose dermique focale tardive type PXE · Elastose dermique focale tardive type pseudoxanthome |
| de | Elastose, dermale fokale, spät-beginnende | PXE-ähnliche fokal-dermale Elastose |
| es | Elastosis dérmica focal de inicio tardío | Elastosis dérmica focal tipo PXE de inicio tardío · Elastosis dérmica focal tipo pseudoxantoma de inicio tardío |
| it | Elastosi dermica focale ad esordio tardivo | Elastosi dermica focale PXE-simile a esordio tardivo · Elastosi dermica focale pseudoxantoma-simile a esordio tardivo |
| nl | Focale dermale elastose met late aanvang | PXE-achtige focale dermale elastose met late aanvang · Pseudoxanthoom-achtige focale dermale elastose met late aanvang |
| pt | Elastose dérmica focal de início tardio | |
| pl | Ogniskowa elastoza skóry o późnym początku | = en |
| cs | Pozdní fokální dermální elastóza | Pozdní fokální dermální elastóza podobná PXE · Pozdní fokální dermální elastóza podobná pseudoxanthoma |
| uk | Пізній вогнищевий дермальний еластоз | Псевдоксантомоподібний пізній вогнищевий фокальний еластоз шкіри · PXE-подібний пізній фокальний дермальний еластоз |
| zh | 迟发性局灶性皮肤弹性组织变性 | 假黄瘤样迟发性局灶性皮肤弹性组织变性 |
| tr | Geç başlangıçlı fokal dermal elastoz | PXE-benzeri geç başlangıçlı fokal dermal elastoz · Psödoksantoma-benzeri geç-başlangıçlı 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 | 0016437 | E | validated | |
| UMLS | C4751167 | 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 | 5 cases | Worldwide | Case(s) | Validated | 15670180[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_15670180[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/228227
- This record as JSON: /data/d/228227.json