Juvenile dermatomyositis
Synonyms: Juvenile DM
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Childhood, Infancy
- Status
- active
An early-onset form of dermatomyositis (DM), a systemic, autoimmune inflammatory muscle disorder with vasculopathy, characterized by proximal and symmetrical muscle weakness, evocative skin lesions, and systemic manifestations. Vasculopathy occurs in the skin, muscle (mainly in the perifascicular area), and sometimes in the intestinal tissue.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Juvenile dermatomyositis | Juvenile DM |
| fr | Dermatomyosite juvénile | DM juvénile |
| de | Dermatomyositis, juvenile | |
| es | Dermatomiositis juvenil | DM juvenil |
| it | Dermatomiosite giovanile | DM giovanile |
| nl | Juveniele dermatomyositis | Juveniele DM |
| pt | Dermatomiosite juvenil | |
| pl | Młodzieńcze zapalenie skórno-mięśniowe | Młodzieńcze DM |
| cs | Juvenilní dermatomyozitida | Juvenilní DM |
| uk | Ювенільний дерматоміозит | Ювенільний ЦД |
| zh | 幼年皮肌炎 | 幼儿DM |
| tr | Juvenil dermatomiyozit | jüvenil DM |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M33.0 | E | specific | validated |
| ICD-11 | 4A41.01 | E | specific | validated |
| MONDO | 0008054 | E | validated | |
| UMLS | C0263666 | E | validated | |
| GARD | 6805 | E | validated | |
| MedDRA | 10008521 | 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 (5):
- ORPHA:329888Juvenile idiopathic inflammatory myopathy
rare neurological diseases; rare systemic and rheumatological diseases - ORPHA:567558Non-genetic systemic disease with glomerulopathy as a major feature
rare renal diseases; rare transplant-related disorders - ORPHA:280373Rare pediatric systemic disease
rare systemic or rheumatologic diseases of childhood - ORPHA:264704Secondary interstitial lung disease specific to childhood associated with a connective tissue disease
rare respiratory diseases; rare transplant-related disorders - ORPHA:290836Systemic disease with skin involvement
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 |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.295 / 100 000 | Worldwide | Value and class | Validated | 12794783[PMID]_7551657[PMID] |
| Point prevalence | Unknown | — | Europe | 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
45 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002960 | Autoimmunity | |
| HP:0003761 | Calcinosis | |
| HP:0000958 | Dry skin | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0003236 | Elevated circulating creatine kinase concentration | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0010783 | Erythema | |
| HP:0012378 | Fatigue | |
| HP:0100579 | Mucosal telangiectasiae | |
| HP:0001324 | Muscle weakness | |
| HP:0003326 | Myalgia | |
| HP:0100614 | Myositis | |
| HP:0100540 | Palpebral edema | |
| HP:0000988 | Skin rash | |
| HP:0100585 | Telangiectasia of the skin | |
| Frequent (79-30%) | ||
| HP:0001596 | Alopecia | |
| HP:0002829 | Arthralgia | |
| HP:0001369 | Arthritis | |
| HP:0002019 | Constipation | |
| HP:0000992 | Cutaneous photosensitivity | |
| HP:0001945 | Fever | |
| HP:0001252 | Hypotonia | |
| HP:0003394 | Muscle spasm | |
| HP:0001029 | Poikiloderma | |
| HP:0000989 | Pruritus | |
| HP:0002091 | Restrictive ventilatory defect | |
| HP:0200042 | Skin ulcer | |
| HP:0002633 | Vasculitis | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0001681 | Angina pectoris | |
| HP:0011675 | Arrhythmia | |
| HP:0011710 | Bundle branch block | |
| HP:0001638 | Cardiomyopathy | |
| HP:0012735 | Cough | |
| HP:0001260 | Dysarthria | |
| HP:0002015 | Dysphagia | |
| HP:0001618 | Dysphonia | |
| HP:0002094 | Dyspnea | |
| HP:0003457 | EMG abnormality | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0001609 | Hoarse voice | |
| HP:0001376 | Limitation of joint mobility | |
| HP:0001701 | Pericarditis | |
| HP:0002206 | Pulmonary fibrosis | |
| HP:0001824 | Weight loss | |
Run the differential on the 12 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/93672
- This record as JSON: /data/d/93672.json