Primary cutaneous plasmacytosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare acquired skin disease characterized by benign proliferation of mature plasma cells with a typical triad of cutaneous lesions, polyclonal hypergammaglobulinemia, and superficial lymphadenopathy, without an apparent underlying cause. The skin lesions consist of multiple round-to-oval, red-to-dark-brown macules, papules, and plaques most commonly found on the trunk, but also the face, neck, and axillae.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous plasmacytosis | |
| fr | Plasmocytose cutanée primitive | |
| de | Primär kutane Plasmazytose | |
| es | Plasmocitosis cutánea primaria | |
| it | Plasmocitosi cutanea primitiva | |
| nl | Primaire cutane plasmocytose | |
| pt | Plasmocitose cutânea primária | |
| pl | Pierwotna plazmacytoza skórna | |
| cs | Primární kožní plazmocytóza | |
| uk | Первинний шкірний плазмоцитоз | |
| zh | 原发性皮肤浆细胞增多症 | |
| tr | Primer kutanöz plazmasitoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L98.6 | NTBT | attributed | validated |
| ICD-11 | EK91.2 | E | specific | validated |
| MONDO | 0018679 | E | validated | |
| UMLS | C4736227 | 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 (2):
- ORPHA:284264IgG4-related disease
rare systemic and rheumatological diseases - ORPHA:90077Other acquired skin disease
rare skin diseases
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 | — | Worldwide | Class only | 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/451602
- This record as JSON: /data/d/451602.json