Confetti-like macular atrophy
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare, acquired, dermis elastic tissue disorder with decreased elastic tissue characterized by multiple, asymptomatic, well demarcated, flat, hypopigmented atrophic macular skin lesions distributed over upper trunk and proximal upper limbs. Histopathological examination reveals atrophic epidermis with decreased basal pigmentation, perivascular mononuclear infiltration in the upper dermis, and disorganized, hyalinized, coarse collagen bundles, and variable loss of elastic fibers in the dermis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Confetti-like macular atrophy | |
| fr | Atrophie maculaire type confetti-like | |
| de | Anetodermie, konfettiartige | |
| es | Atrofia macular en confetti | |
| it | Atrofia maculare simil-coriandoli | |
| nl | Confetti-achtige macula-atrofie | |
| pt | Atrofia macular confetti-like | |
| pl | Zanik plamki podobny do konfetti | |
| cs | Makulární atrofie podobná konfetám | |
| uk | Конфетті-подібна макулярна атрофія | |
| zh | 彩色纸屑样斑状萎缩 | |
| tr | Konfeti benzeri maküler atrofi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L90.8 | NTBT | attributed | validated |
| ICD-11 | EE41.1 | NTBT | attributed | validated |
| MONDO | 0016376 | E | validated | |
| UMLS | C5687155 | 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 | 2 cases | Worldwide | Case(s) | Validated | 19878392[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_19878392[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/221142
- This record as JSON: /data/d/221142.json