Orphanet Rare Disease Ontology

Last uploaded: December 13, 2023
Preferred Name

Juvenile dermatomyositis

Synonyms

Juvenile DM

Definitions

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.

ID

http://www.orpha.net/ORDO/Orphanet_93672

alternative_term

Juvenile DM

definition

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.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=93672

has_age_of_onset

Adolescent

Childhood

Infancy

has_inheritance

Not applicable

hasDbXref

UMLS:C0263666

MedDRA:10008521

ICD-10:M33.0

ICD-11:4A41.01

label

Juvenile dermatomyositis

notation

ORPHA:93672

part_of

http://www.orpha.net/ORDO/Orphanet_329888

http://www.orpha.net/ORDO/Orphanet_264704

http://www.orpha.net/ORDO/Orphanet_567558

http://www.orpha.net/ORDO/Orphanet_280373

http://www.orpha.net/ORDO/Orphanet_290836

prefixIRI

ORDO:Orphanet_93672

prefLabel

Juvenile dermatomyositis

present_in

Worldwide AND has_annual_incidence_average_value : 0.295 AND has_annual_incidence_range : 1-9 / 1 000 000

Europe AND has_point_prevalence_range : Unknown

treeView

http://www.orpha.net/ORDO/Orphanet_329888

http://www.orpha.net/ORDO/Orphanet_264704

http://www.orpha.net/ORDO/Orphanet_567558

http://www.orpha.net/ORDO/Orphanet_280373

http://www.orpha.net/ORDO/Orphanet_290836

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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