Childhood Type Dermatomyositis malady

Rare diseases, Neuronal diseases, Nephrological diseases, Respiratory diseases, Bone diseases, Skin diseases categories

Aliases & Classifications for Childhood Type Dermatomyositis

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30LifeMap Discovery®, 8Disease Ontology, 42NIH Rare Diseases, 61UMLS, 48Orphanet, 33MeSH, 39NCIt, 56SNOMED-CT, 26ICD10 via Orphanet, 34MESH via Orphanet, 62UMLS via Orphanet, 25ICD10
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Aliases & Descriptions for Childhood Type Dermatomyositis:

Name: Childhood Type Dermatomyositis 30 8
Juvenile Dermatomyositis 8 42 48 61
Rhizomelic Pseudopolyarthritis 42 61
Polymyalgia Rheumatica 42 61
Juvenile Polymyositis 42 48
Dermatomyositis 42 61
Juvenile Dm 42 48
Dermatomyositis, Childhood Type 61
Adult Type Dermatomyositis 61
Childhood Dermatomyositis 8
Giant Cell Arteritis 61
Juvenile Myositis 42
Juvenile Pm 48
Jpm 42
Jdm 42
Dm 42


Characteristics (Orphanet epidemiological data):

juvenile dermatomyositis:
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Worldwide); Age of onset: Adolescent,Childhood,Infancy; Age of death: normal life expectancy

External Ids:

Disease Ontology8 DOID:14203
MeSH33 D003882
NCIt39 C27576
Orphanet48 93568, 93672
SNOMED-CT56 201446001, 1212005
ICD10 via Orphanet26 M33.2, M33.0
MESH via Orphanet34 C538250
UMLS via Orphanet62 C0263666, C2931785
ICD1025 M33.0

Summaries for Childhood Type Dermatomyositis

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NIH Rare Diseases:42 Juvenile dermatomyositis has some similarities to adult dermatomyositis and polymyositis. it typically affects children ages 2 to 15 years, with symptoms that include weakness of the muscles close to the trunk of the body, inflammation, edema, muscle pain, fatigue, skin rashes, abdominal pain, fever, and contractures. children with juvenile dermatomyositis may have difficulty swallowing and breathing, and the heart may also be affected.  about 20 to 30 percent of children with juvenile dermatomyositis develop calcium deposits in the soft tissue. affected children may not show higher than normal levels of the muscle enzyme creatine kinase in their blood but have higher than normal levels of other muscle enzymes. last updated: 5/31/2011

MalaCards based summary: Childhood Type Dermatomyositis, also known as juvenile dermatomyositis, is related to polymyalgia rheumatica and dermatomyositis, and has symptoms including chondrocalcinosis, dry skin and skin rash. The drugs immunoglobulins, intravenous and potassium aminobenzoate have been mentioned in the context of this disorder. Affiliated tissues include uterus, skin and heart.

Related Diseases for Childhood Type Dermatomyositis

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Graphical network of the top 20 diseases related to Childhood Type Dermatomyositis:

Diseases related to childhood type dermatomyositis

Symptoms for Childhood Type Dermatomyositis

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 48 (show all 44)
  • palpebral edema/periorbital edema
  • dry/squaly skin/exfoliation
  • cutaneous rash
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • telangiectasiae of the skin
  • telangiectasiae of mucosae
  • subcutaneous nodules/lipomas/tumefaction/swelling
  • muscle weakness/flaccidity
  • myalgia/muscular pain
  • abnormal muscle biopsy/muscle enzymes/cpk/ldh/aldolase/creatin phosphokinase
  • periarticular tissue anomaly/extraarticular calcifications
  • autoimmunity/autoimmune reaction/autoantibodies
  • asthenia/fatigue/weakness
  • biological inflammatory syndrome/increased erythrocyte sedimentation rate/crp
  • chronic skin infection/ulcerations/ulcers/cancrum
  • skin photosensitivity
  • follicular/erythematous/edematous papules/milium
  • pruritus/itching
  • telangiectasic erythema/poikiloderma
  • alopecia
  • constipation
  • interstitial/restrictive pneumopathy/restrictive respiratory syndrome
  • vascularitis/vasculitides/arteritis
  • hypotonia
  • contractures/cramps/trismus/tetania/claudication/opisthotonos
  • articular/joint pain/arthralgia
  • arthritis/synovitis/synovial proliferation
  • fever/chilling
  • feeding disorder/dysphagia/swallowing/sucking disorder/esophageal dyskinesia
  • acute abdominal pain/colic
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • lung fibrosis
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • cough
  • abnormal cry/voice/phonation disorder/nasal speech
  • cardiomyopathy/hypertrophic/dilated
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • cardiac rhythm disorder/arrhythmia
  • cardiac conduction defect/sinoauricular/heart/auriculoventricular/branch block
  • angor pectoris/myocardial infarction
  • elocution disorders/dysarthria/dysphonia
  • abnormal emg/electromyogram/electropmyography
  • restricted joint mobility/joint stiffness/ankylosis
  • weight loss/loss of appetite/break in weight curve/general health alteration

HPO human phenotypes related to Childhood Type Dermatomyositis:

(show all 36)
id Description Frequency HPO Source Accession
1 chondrocalcinosis hallmark (90%) HP:0000934
2 dry skin hallmark (90%) HP:0000958
3 skin rash hallmark (90%) HP:0000988
4 muscle weakness hallmark (90%) HP:0001324
5 autoimmunity hallmark (90%) HP:0002960
6 myalgia hallmark (90%) HP:0003326
7 periorbital edema hallmark (90%) HP:0100539
8 mucosal telangiectasiae hallmark (90%) HP:0100579
9 teleangiectasia of the skin hallmark (90%) HP:0100585
10 pruritus typical (50%) HP:0000989
11 cutaneous photosensitivity typical (50%) HP:0000992
12 poikiloderma typical (50%) HP:0001029
13 muscular hypotonia typical (50%) HP:0001252
14 arthritis typical (50%) HP:0001369
15 flexion contracture typical (50%) HP:0001371
16 alopecia typical (50%) HP:0001596
17 constipation typical (50%) HP:0002019
18 restrictive lung disease typical (50%) HP:0002091
19 vasculitis typical (50%) HP:0002633
20 arthralgia typical (50%) HP:0002829
21 abnormality of temperature regulation typical (50%) HP:0004370
22 skin ulcer typical (50%) HP:0200042
23 limitation of joint mobility occasional (7.5%) HP:0001376
24 abnormality of the voice occasional (7.5%) HP:0001608
25 hypertrophic cardiomyopathy occasional (7.5%) HP:0001639
26 coronary artery disease occasional (7.5%) HP:0001677
27 abnormality of the pericardium occasional (7.5%) HP:0001697
28 weight loss occasional (7.5%) HP:0001824
29 abdominal pain occasional (7.5%) HP:0002027
30 respiratory insufficiency occasional (7.5%) HP:0002093
31 neurological speech impairment occasional (7.5%) HP:0002167
32 pulmonary fibrosis occasional (7.5%) HP:0002206
33 gastrointestinal hemorrhage occasional (7.5%) HP:0002239
34 emg abnormality occasional (7.5%) HP:0003457
35 feeding difficulties in infancy occasional (7.5%) HP:0008872
36 arrhythmia occasional (7.5%) HP:0011675

Drugs & Therapeutics for Childhood Type Dermatomyositis

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Drug clinical trials:

Search ClinicalTrials for Childhood Type Dermatomyositis

Search NIH Clinical Center for Childhood Type Dermatomyositis

Inferred drug relations via UMLS61/NDF-RT40:

Genetic Tests for Childhood Type Dermatomyositis

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Anatomical Context for Childhood Type Dermatomyositis

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MalaCards organs/tissues related to Childhood Type Dermatomyositis:

Uterus, Skin, Heart, Lung, Brain, Breast, Bone, Liver, Myeloid, T cells

Animal Models for Childhood Type Dermatomyositis or affiliated genes

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Publications for Childhood Type Dermatomyositis

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Variations for Childhood Type Dermatomyositis

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Expression for genes affiliated with Childhood Type Dermatomyositis

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LifeMap Discovery
Genes differentially expressed in tissues of Childhood Type Dermatomyositis patients vs. healthy controls: 30 (show all 33)
id Gene Description Tissue Up/Dn Fold Change (log2) P value
2KRT1keratin 1Uterus-5.320.000
3UPK1Auroplakin 1AUterus-4.790.000
4MALmal, T-cell differentiation proteinUterus-4.730.000
5KRT4keratin 4Uterus-4.450.000
6SPRR3small proline-rich protein 3Uterus-4.430.000
7DSG1desmoglein 1Uterus-4.270.000
8CDKN2Acyclin-dependent kinase inhibitor 2AUterus+4.210.000
9CRISP3cysteine-rich secretory protein 3Uterus-4.130.000
10CRCT1cysteine-rich C-terminal 1Uterus-3.920.000
11SPINK5serine peptidase inhibitor, Kazal type 5Uterus-3.670.000
12SLURP1secreted LY6/PLAUR domain containing 1Uterus-3.540.000
13APOC1apolipoprotein C-IUterus+3.530.000
14HOPXHOP homeoboxUterus-3.510.000
15EDN3endothelin 3Uterus-3.480.000
16FANCIFanconi anemia, complementation group IUterus+3.420.000
17SYNGR3synaptogyrin 3Uterus+3.410.000
18CRYABcrystallin, alpha BUterus-3.390.000
19KRT13keratin 13Uterus-3.350.004
20MCM5minichromosome maintenance complex component 5Uterus+3.310.000
21ALOX12arachidonate 12-lipoxygenaseUterus-3.260.000
22PPP1R3Cprotein phosphatase 1, regulatory subunit 3CUterus-3.230.000
25NUSAP1nucleolar and spindle associated protein 1Uterus+3.170.000
27IL1R2interleukin 1 receptor, type IIUterus-3.140.000
28CFDcomplement factor D (adipsin)Uterus-3.130.000
29SOSTDC1sclerostin domain containing 1Uterus-3.080.000
30ESR1estrogen receptor 1Uterus-3.060.000
31PAMR1peptidase domain containing associated with muscle regeneration 1Uterus-3.060.000
32ENDOUendonuclease, polyU-specificUterus-3.040.000
33HOXC6homeobox C6Uterus+3.010.000

Search GEO for disease gene expression data for Childhood Type Dermatomyositis.

Pathways for genes affiliated with Childhood Type Dermatomyositis

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Compounds for genes affiliated with Childhood Type Dermatomyositis

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GO Terms for genes affiliated with Childhood Type Dermatomyositis

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Sources for Childhood Type Dermatomyositis

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26ICD10 via Orphanet
34MESH via Orphanet
47OMIM via Orphanet
57SNOMED-CT via Orphanet
62UMLS via Orphanet