Scleromyxedema without monoclonal gammopathy
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
Scleromyxedema without monoclonal gammopathy is a form of atypical lichen myxedematosus, characterized by a generalized sclerodermoid infiltration of skin studded with multiple, firm papules of 1-3 mm in diameter involving face (leonine appearance), trunk, and limbs, without monoclonal gammopathy. The involvement of the face can be missing and pruritus may be prominent.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Scleromyxedema without monoclonal gammopathy | |
| fr | Scléromyxoedème sans gammopathie monoclonale | |
| de | Skleromyxödem ohne monoklonale Gammopathie | |
| es | Escleromixedema sin gammapatía monoclonal | |
| it | Scleromixedema senza gammopatia monoclonale | |
| nl | Scleromyxoedeem zonder monoklonale gammopathie | |
| pt | Escleromixedema sem gamopatia monoclonal | |
| pl | Liszaj śluzowaty twardzinowy bez gammopatii monoklonalnej | |
| cs | Skleromyxedém bez monoklonální gamapatie | |
| uk | Склеромікседема без моноклональної гамопатії | |
| zh | 硬化性粘液水肿不伴单克隆丙种球蛋白病 | |
| tr | Monoklonal gammopati olmayan skleromiksödem |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L98.5 | NTBT | attributed | validated |
| ICD-11 | EB90.11 | NTBT | attributed | validated |
| MONDO | 0019585 | E | validated | |
| UMLS | C5681463 | 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:86797Atypical lichen myxedematosus
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 15 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| 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/90400
- This record as JSON: /data/d/90400.json