Nodular cutaneous amyloidosis
Synonyms: PLCNA · Primary localized cutaneous nodular amyloidosis
- Type
- Disease
- Group
- Disorder
- Status
- active
Primary localized cutaneous nodular amyloidosis (PLCNA) is the most rare form of primary cutaneous amyloidosis, a skin disease characterized by the accumulation of amyloid deposits in the dermis, characterized clinically by yellowish waxy crusted nodules and papules on the face, lower extremities, trunk, scalp, and genitalia and histologically by the localized deposition of immunoglobulin-derived amyloid in the papillary dermis and subcutis. PLCNA can be associated with connective tissue disorders such as Sjögren's syndrome and CREST syndrome.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Nodular cutaneous amyloidosis | PLCNA · Primary localized cutaneous nodular amyloidosis |
| fr | Amylose cutanée nodulaire | Amylose cutanée nodulaire primitive localisée · PLCNA |
| de | Noduläre kutane Amyloidose | Amyloidose, kutane lokalisierte noduläre primäre · NPLCA · PLCNA |
| es | Amiloidosis cutánea nodular | Amiloidosis nodular primaria cutánea localizada · PLCNA |
| it | Amiloidosi cutanea nodulare | Amiloidosi nodulare cutanea localizzata primitiva · PLCNA |
| nl | Nodulaire cutane amyloïdose | PLCNA · Primaire gelokaliseerde cutane nodulaire amyloïdose |
| pt | Amiloidose cutânea, forma nodular | |
| pl | Guzkowata amyloidoza skórna | Pierwotna zlokalizowana guzkowata amyloidoza skórna · plCNA |
| cs | Nodulární kožní amyloidóza | PLCNA · Primární nodulární amyloidóza lokalizovaná na kůži |
| uk | Вузликовий шкірний амілоїдоз | PLCNA · Первинний локалізований шкірний вузликовий амілоїдоз |
| zh | 结节性皮肤淀粉样变 | 原发性皮肤结节性淀粉样变性 |
| tr | Nodüler kutanöz amiloidoz | PLCNA · Primer lokalize kutanöz nodüler amiloidoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E85.4+ | NTBT | attributed | validated |
| ICD-10 | L99.0* | NTBT | attributed | validated |
| ICD-11 | 5D00.0 | NTBT | index | validated |
| MONDO | 0015302 | E | validated | |
| UMLS | C4274331 | E | validated | |
| GARD | 10562 | E | validated | |
| MedDRA | 10056953 | 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:137807Primary cutaneous amyloidosis
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/137810
- This record as JSON: /data/d/137810.json