Papular elastorrhexis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare, acquired, dermis elastic tissue disorder characterized by multiple, asymptomatic, firm, well-demarcated, nonfollicular, hypopigmented or skin-colored papules, with a diameter of less than 1 cm, distributed symmetrically over trunk and/or proximal limbs (rarely, head, neck, shoulders, armpits, thighs), with no extracutaneous manifestations. Histopathology typically reveals decreased and fragmented elastic fibers, thickened and/or homogenized collagen bundles and, in some, a mild, perivascular, lymphocytic infiltrate in the dermis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Papular elastorrhexis | |
| fr | Elastorrhexie papuleuse | |
| de | Elastorrhexis, papulöse | Naevus anelasticus, disseminierter · Nevus anelasticus |
| es | Elastorrexis papular | |
| it | Elastoressi papulosa | Collagenoma eruttivo · Nevo anelastico · Nevo anelastico disseminato |
| nl | Papula-elastorrhexis | |
| pt | Elastorrhexis papular | |
| pl | Grudkowa fragmentacja włókien elastyny | Eruptive collagenoma Wyrzutowa kolagenoma · Rozsiane znamię ze zwyrodnieniem włókien sprężystych · Znamię ze zwyrodnieniem włókien sprężystych |
| cs | Papulární elastorrhexis | Diseminovaný nevus anelasticus · Eruptivní kolagenom · Nevus anelasticus |
| uk | Папульозний еласторексис | |
| zh | 丘疹性弹性组织破裂 | |
| tr | Papüler elastoreksis |
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 | 0016443 | E | validated | |
| UMLS | C5680907 | 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 | <1 / 1 000 000 | — | Europe | 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/228264
- This record as JSON: /data/d/228264.json