loading...| Preferred Name |
juvenile dermatomyositis |
|
| Synonyms |
myoseptum inflammation childhood type dermatomyositis (disorder) Dermatomyositis, Childhood Type juvenile dermatomyositis Dermatomyositides, Childhood Type Childhood Type Dermatomyositides childhood dermatomyositis myoseptumitis juvenile DM inflammation of myoseptum childhood Dermatomyositis Childhood Type Dermatomyositis juvenile myositis childhood type dermatomyositis myopathy, familial idiopathic inflammatory JDM JPM myositis |
|
| Definitions |
A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6) Juvenile dermatomyositis (JDM) is the early-onset form of dermatomyositis (DM), a systemic, autoimmune inflammatory muscle disorder, characterized by proximal muscle weakness, evocative skin lesion, and systemic manifestations. |
|
| ID |
http://www.ebi.ac.uk/efo/EFO_0000557 |
|
| closeMatch | ||
| database_cross_reference |
NCIt:C27576 NCIT:C27576 MESH:C538250 UMLS:C2931785 SNOMEDCT:1212005 MESH:C000598745 Orphanet:93672 icd11.foundation:1428089375 MedDRA:10008521 SCTID:1212005 NANDO:2200418 MedDRA:10078425 OMIM:160750 DOID:14203 GARD:6805 MONDO:0008054 |
|
| definition |
A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6) Juvenile dermatomyositis (JDM) is the early-onset form of dermatomyositis (DM), a systemic, autoimmune inflammatory muscle disorder, characterized by proximal muscle weakness, evocative skin lesion, and systemic manifestations. |
|
| disease has inflammation site | ||
| exactMatch |
http://purl.obolibrary.org/obo/NCIT_C27576 http://purl.obolibrary.org/obo/Orphanet_93672 |
|
| gwas_trait |
true |
|
| has_exact_synonym |
myoseptum inflammation childhood type dermatomyositis (disorder) Dermatomyositis, Childhood Type juvenile dermatomyositis Dermatomyositides, Childhood Type Childhood Type Dermatomyositides childhood dermatomyositis myoseptumitis juvenile DM inflammation of myoseptum childhood Dermatomyositis Childhood Type Dermatomyositis |
|
| has_related_synonym |
juvenile myositis childhood type dermatomyositis myopathy, familial idiopathic inflammatory JDM JPM myositis |
|
| id |
EFO:0000557 |
|
| in_subset |
http://purl.obolibrary.org/obo/mondo/mondo-base#rare http://purl.obolibrary.org/obo/mondo/mondo-base#otar http://purl.obolibrary.org/obo/mondo/mondo-base#gard_rare http://purl.obolibrary.org/obo/mondo/mondo-base#orphanet_rare http://purl.obolibrary.org/obo/mondo/mondo-base#nord_rare http://purl.obolibrary.org/obo/mondo/mondo-base#ordo_disease |
|
| label |
juvenile dermatomyositis |
|
| notation |
EFO:0000557 |
|
| prefLabel |
juvenile dermatomyositis |
|
| see also |
https://rarediseases.info.nih.gov/diseases/6805/juvenile-dermatomyositis |
|
| term editor |
James Malone |
|
| subClassOf |
http://www.ebi.ac.uk/efo/EFO_0000398 http://purl.obolibrary.org/obo/MONDO_0018010 |