Neonatal dermatomyositis
Synonyms: Neonatal DM
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Neonatal
- Status
- active
A rare secondary neonatal autoimmune disease characterized by generalized weakness, severe hypotonia, absent or reduced deep tendon reflexes, and highly elevated serum creatine kinase levels presenting in the neonatal period. Perifascicular atrophy in the presence of a diffuse perivascular inflammatory cell exudate is observed on muscle biopsy.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neonatal dermatomyositis | Neonatal DM |
| fr | Dermatomyosite néonatale | DM néonatale |
| de | Dermatomyositis, neonatale | Neonatale DM |
| es | Dermatomiositis neonatal | DM neonatal |
| it | Dermatomiosite neonatale | DM neonatale |
| nl | Neonatale dermatomyositis | Neonatale DM |
| pt | Dermatomiosite neonatal | |
| pl | Zapalenie skórno-mięśniowe noworodków | DM noworodków |
| cs | Neonatální dermatomyozitida | Neonatální DM |
| uk | Неонатальний дерматоміозит | Неонатальний DM |
| zh | 新生儿皮肌炎 | 新生儿DM |
| tr | Yenidoğan dermatomiyoziti | Yenidoğan DM |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M33.1 | NTBT | attributed | validated |
| ICD-11 | 4A41.0 | NTBT | attributed | validated |
| MONDO | 0018359 | E | validated | |
| UMLS | C4751516 | 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 | 3 cases | Worldwide | Case(s) | Validated | 3508696[PMID]_8888050[PMID]_3508696[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_3508696[PMID]_8888050[PMID]_3508696[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/398117
- This record as JSON: /data/d/398117.json