Scleroderma
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Scleroderma is a rare autoimmune connective tissue disorder characterized by abnormal hardening of the skin and, sometimes, other organs. It is classified into two main forms: localized scleroderma and systemic sclerosis (SSc), the latter comprising three subsets; diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc) and limited SSc (lSSc).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Scleroderma | |
| fr | Sclérodermie | |
| de | Sklerodermie | |
| es | Esclerodermia | |
| it | Sclerodermia | |
| nl | Sclerodermie | |
| pt | Esclerodermia | |
| pl | Twardzina | |
| cs | Sklerodermie | |
| uk | Склеродермія | |
| zh | 硬皮病 | |
| tr | Skleroderma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0019340 | E | validated | |
| MONDO | 19340 | E | validated | |
| UMLS | C0011644 | E | validated | |
| MedDRA | 10039710 | 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:290836Systemic disease with skin involvement
rare skin diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
5 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 1.41 / 100 000 | Worldwide | Value and class | Validated | [EXPERT] |
| Point prevalence | 1-5 / 10 000 | 42 / 100 000 | Worldwide | Value and class | Validated | [EXPERT] |
| Annual incidence | 1-9 / 100 000 | 1.5 / 100 000 | Australia | Value and class | Validated | 11456035[PMID] |
| Point prevalence | 1-5 / 10 000 | 23 / 100 000 | Australia | Value and class | Validated | 11456035[PMID] |
| Point prevalence | >1 / 1000 | 469 / 100 000 | Specific population | Value and class | Validated | 8702445[PMID] |
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/801
- This record as JSON: /data/d/801.json