Primary cutaneous amyloidosis
Synonyms: PLCA · Primary localized cutaneous amyloidosis
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Autosomal dominant, Not applicable
- Age of onset
- All ages
- Status
- active
A rare group of skin diseases characterized histologically by the extracellular accumulation of amyloid deposits in the dermis. Rare forms include lichen amyloidosus, X-linked reticulate pigmentary disorder, primary localized cutaneous nodular amyloidosis, and macular amyloidosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous amyloidosis | PLCA · Primary localized cutaneous amyloidosis |
| fr | Amylose cutanée primitive | PLCA |
| de | Primäre kutane Amyloidose | Amyloidose, kutane lokalisierte primäre · PLCA |
| es | Amiloidosis cutánea primaria | Amiloidosis cutánea primaria localizada · PLCA |
| it | Amiloidosi cutanea primitiva | Amiloidosi cutanea localizzata primitiva · PLCA |
| nl | Primaire cutane amyloïdose | PLCA · Primaire gelokaliseerde cutane amyloïdose |
| pt | Amiloidose cutânea | |
| pl | Pierwotna amyloidoza skórna | Pierwotna zlokalizowana amyloidoza skórna · plCA |
| cs | Primární kožní amyloidóza | PLCA · Primární lokalizovaná kožní amyloidóza |
| uk | Первинний шкірний амілоїдоз | PLCA · Первинний локалізований шкірний амілоїдоз |
| zh | 原发性皮肤淀粉样变 | 原发性皮肤淀粉样变病 |
| tr | Primer kutanöz amiloidoz | PLCA · Primer lokalize kutanöz amiloidoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | 5D00.0 | NTBT | index | validated |
| MONDO | 0015301 | E | validated | |
| MONDO | 15301 | E | validated | |
| MeSH | C562642 | E | validated | |
| UMLS | C0268397 | E | validated | |
| GARD | 132 | E | validated | |
| MedDRA | 10011659 | 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):
- ORPHA:79381Other dermis disorder
rare skin diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 9.8 / 100 000 | Taiwan, Province of China | Value and class | Validated | 21070198[PMID] |
| 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/137807
- This record as JSON: /data/d/137807.json