Scleredema
Synonyms: Buschke scleredema
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Infancy
- Status
- active
A rare acquired skin disease characterized by excessive mucin deposition and thickened collagen bundles in the dermis, resulting in woody, non-pitting induration of the skin of the neck, spreading to the shoulders and upper trunk, but sparing hands and feet. According to the association with preceding or underlying conditions, three types can be distinguished: type 1 usually follows a febrile infection, type 2 is associated with paraproteinemia, and type 3 occurs in patients with diabetes mellitus. Especially in types 2 and 3, extracutaneous involvement may be present. Other potentially associated conditions include a variety of endocrinopathies, systemic diseases, and neoplasms.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Scleredema | Buschke scleredema |
| fr | Scléroedème | Scléroedème de Buschke |
| de | Sklerödem | Sklerödem Buschke |
| es | Escleredema | Escleredema de Buschke |
| it | Scleredema | Scleredema di Buschke |
| nl | Scleroedeem | Scleroedeem van Buschke |
| pt | Escleredema | |
| pl | Twardzina obrzękowa (scleredema) | Twardzina obrzękowa Buschkego |
| cs | Skleredém | Scleredema adultorum Buschke |
| uk | Склередема | Склеродермія Бушке |
| zh | 硬化病 | Buschke硬肿病 |
| tr | Sklerödem | Buschke sklerödem |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M34.8 | NTBT | index | validated |
| ICD-11 | EE7Y | NTBT | index | validated |
| MONDO | 0006606 | E | validated | |
| MONDO | 6606 | E | validated | |
| UMLS | C0036413 | E | validated | |
| GARD | 5975 | E | validated | |
| MedDRA | 10055953 | 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: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 | 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/352763
- This record as JSON: /data/d/352763.json