Neonatal scleroderma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Neonatal
- Status
- active
A rare secondary neonatal autoimmune disease characterized by neonatal-onset of erythematous skin lesions with a linear appearance that gradually become indurated and hyperpigmented and progressively present skin atrophy. Positive serum antibodies (in particular antinuclear antibodies and/or rheumatoid factor) may be associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neonatal scleroderma | |
| fr | Sclérodermie néonatale | |
| de | Neonatale Sklerodermie | |
| es | Escleroderma neonatal | |
| it | Sclerodermia neonatale | |
| nl | Neonatale sclerodermie | |
| pt | Esclerodermia neonatal | |
| pl | Twardzina noworodków | |
| cs | Neonatální sklerodermie | |
| uk | Неонатальна склеродермія | |
| zh | 新生儿硬皮病 | |
| tr | Yenidoğan skleroderması |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | P83.8 | NTBT | inclusion | validated |
| ICD-11 | KC22.2 | NTBT | attributed | validated |
| MONDO | 0018361 | E | validated | |
| UMLS | C4509425 | 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:398091Secondary neonatal autoimmune disease
rare systemic and rheumatological diseases; rare systemic or rheumatologic diseases of childhood
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 | 6 cases | Worldwide | Case(s) | Validated | 16887444[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_16887444[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/398127
- This record as JSON: /data/d/398127.json