Cutaneous mastocytosis
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
A rare group of mastocytosis diseases characterized by abnormal accumulation and proliferation of mast cells in the skin and including the three recognised forms: diffuse cutaneous mastocytosis, cutaneous mastocytoma and, the most common form, maculopapular cutaneous mastocytosis. In some cases (most commonly in adults), cutaneous mastocytosis may occur in association with mast cell infiltration of various extracutaneous organs, in which case the disorder is referred to as systemic mastocytosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Cutaneous mastocytosis | |
| fr | Mastocytose cutanée | |
| de | Mastozytose, kutane | |
| es | Mastocitosis cutánea | |
| it | Mastocitosi cutanea | |
| nl | Cutane mastocytose | |
| pt | Mastocitose cutânea | |
| pl | Mastocytoza skórna | |
| cs | Kožní mastocytóza | |
| uk | Шкірний мастоцитоз | |
| zh | 皮肤肥大细胞增生病 | |
| tr | Kutanöz mastositoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | 2A21.1 | E | specific | validated |
| MONDO | 0019023 | E | validated | |
| MONDO | 19023 | E | validated | |
| MeSH | D034701 | E | validated | |
| UMLS | C1136033 | E | validated | |
| GARD | 7842 | 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:98292Mastocytosis
rare hematological diseases; rare neoplastic diseases - ORPHA:90077Other acquired skin disease
rare skin diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | 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/66646
- This record as JSON: /data/d/66646.json