Mycosis fungoides and variants
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A group of cutaneous T-cell lymphomas characterized by the indolent, slow progression of patches, plaques, and tumors. There are four known variants: classic mycosis fungoides, folliculotropic mycosis fungoides, localized pagetoid reticulosis and granulomatous slack skin. Classic MF is characterized by slow progression from patches on sun-protracted areas to more infiltrated plaques and tumors; folliculotropic MF preferentially involves the head and neck region; localized pagetoid reticulosis shows localized patches or plaques, usually involving one extremity; granulomatous slack skin is extremely rare and characterized by the presence of circumscribed areas of pendulous lax skin.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Mycosis fungoides and variants | |
| fr | Mycosis fongoïde et variants | |
| de | Mycosis fungoides-Varianten | |
| es | Micosis fungoide y variantes | |
| it | Micosi fungoide e varianti | |
| nl | Mycosis fungoides en varianten | |
| pt | Micose fungóide e variantes | |
| pl | Ziarniniak grzybiasty i warianty | |
| cs | Mycosis fungoides a klinické varianty | |
| uk | Фунгоїдні мікози та їх різновиди | |
| zh | 蕈样真菌病及其变种 | |
| tr | Mikoz fungoides ve çeşitleri |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C84.0 | E | specific | validated |
| ICD-11 | 2B01 | E | specific | validated |
| MONDO | 0015821 | E | validated | |
| MONDO | 15821 | E | validated | |
| UMLS | C1513782 | 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:178548Indolent primary cutaneous T-cell lymphoma
rare skin diseases; rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.59 / 100 000 | Europe | Value and class | Validated | ORPHANET |
| 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/178566
- This record as JSON: /data/d/178566.json