DM
MCID: DRM010
MIFTS: 70

Dermatomyositis (DM) malady

Neuronal diseases, Nephrological diseases, Bone diseases, Skin diseases categories

Summaries for Dermatomyositis

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 63Wikipedia, 46OMIM, 32MalaCards
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NIH Rare Diseases:42 Dermatomyositis is one of a group of acquired muscle diseases called inflammatory myopathies (disorder of muscle tissue or muscles), which are characterized by chronic muscle inflammation accompanied by muscle weakness. the cardinal symptom is a skin rash that precedes or accompanies progressive muscle weakness. dermatomyositis may occur at any age, but is most common in adults in their late 40s to early 60s, or children between 5 and 15 years of age. there is no cure for dermatomyositis, but the symptoms can be treated. options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest. the cause of dermatomyositis is unknown. last updated: 8/26/2013

MalaCards: Dermatomyositis, also known as polymyositis with skin involvement, is related to adult dermatomyositis and interstitial lung disease, and has symptoms including cardiac rhythm disorder/arrhythmia, angor pectoris/myocardial infarction and vascularitis/vasculitides/arteritis. An important gene associated with Dermatomyositis is IFIH1 (interferon induced with helicase C domain 1), and among its related pathways are Immune System and Interferon Signaling. The compounds pyrophosphate and Adenosine triphosphate have been mentioned in the context of this disorder. Affiliated tissues include muscle or, skin and lung.

Disease Ontology:8 A myositis that results in inflammation located in muscle or located in skin where a skin rash is often seen prior to the onset of muscle weakness. the disease may result from either a viral infection or an autoimmune reaction.

NINDS:43 Dermatomyositis is one of a group of muscle diseases known as the inflammatory myopathies, which are characterized by chronic muscle inflammation accompanied by muscle weakness.

Wikipedia:63 Dermatomyositis (DM) is a connective-tissue disease related to polymyositis (PM) that is characterized... more...

Description from OMIM:46 160750

Aliases & Classifications for Dermatomyositis

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 10DISEASES, 44Novoseek, 48Orphanet, 46OMIM, 60UMLS, 56SNOMED-CT, 39NCIt, 27ICD9CM, 34MeSH, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet, 35MESH via Orphanet, 26ICD10 via Orphanet, 25ICD10
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Classifications:



Characteristics (Orphanet epidemiological data):

48
dermatomyositis:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Variable


Aliases & Descriptions:

dermatomyositis 8 42 43 10 44 48 46
polymyositis with skin involvement 8
dermatopolymyositis, unspecified 8
adult-onset dermatomyositis 48
adult type dermatomyositis 60
dermatopolymyositis 8
dm 48


External Ids:

Disease Ontology8 DOID:10223
NCIt39 C26744
ICD9CM27 710.3
SNOMED-CT via Orphanet57 396230008, 402425006, 396229003
UMLS via Orphanet61 C0011633
MESH via Orphanet35 D003882
ICD10 via Orphanet26 M33.0, M33.1
OMIM46 160750
ICD1025 M33, M33.9

Related Diseases for Dermatomyositis

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Sources:
17GeneCards, 18GeneDecks
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Diseases in the Dermatomyositis family:

Adult Dermatomyositis

Diseases related to Dermatomyositis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 273)
idRelated DiseaseScoreTop Affiliating Genes
1adult dermatomyositis31.2IFIT3, UBA2
2interstitial lung disease31.1SFTPD, HARS
3myositis31.0ICAM1, HARS, PIK3C2A, CHKB, VCAM1, GARS
4pneumonia30.7PIK3C2A, ICAM1, SFTPD
5inclusion body myositis30.7VCAM1, ICAM1
6myopathy30.6PIK3C2A, CHKB, GARS
7systemic lupus erythematosus30.6ICAM1, VCAM1, CCR7, HLA-DQA1
8diabetes mellitus30.6IFIH1, ICAM1, HLA-DQA2, HLA-DQA1, PIK3C2A, VCAM1
9arthritis30.6ICAM1, CCR7
10melanoma30.5IFIH1, CCR7, ICAM1, CHKB, VCAM1
11rheumatoid arthritis30.4SFTPD, ICAM1, CHKB, CCR7, VCAM1
12connective tissue disease30.2VCAM1, ICAM1
13dermatitis30.0HARS, ICAM1, PIK3C2A, VCAM1
14influenza30.0CCR7, IFIH1, SFTPD
15psoriasis30.0VCAM1, ICAM1, CCR7
16tuberculosis30.0CCR7, SFTPD
17celiac disease29.8HLA-DQA1, HLA-DQA2
18polymyositis29.7ICAM1, CCR7, SFTPD, HARS, PIK3C2A, CHKB
19sarcoidosis29.6SFTPD, ICAM1
20respiratory failure29.6SFTPD, ICAM1, PIK3C2A
21calcinosis10.7
22lupus erythematosus10.5
23myotonic dystrophy10.4
24childhood type dermatomyositis10.4
25scleroderma10.4
26skin disease10.4
27panniculitis10.3
28amyopathic dermatomyositis10.3
29breast cancer10.2
30systemic scleroderma10.2
31adenocarcinoma10.2
32maturity-onset diabetes of the young10.2
33multicentric reticulohistiocytosis10.2
34pneumatosis cystoides intestinalis10.2
35fasciitis10.2
36lipodystrophy10.2
37testicular cancer10.2
38pityriasis rubra pilaris10.1
39nasopharyngitis10.1
40bladder carcinoma10.1
41vasculitis10.1
42diabetes insipidus10.1
43acute interstitial pneumonia10.1
44cutaneous lupus erythematosus10.1
45pemphigus10.1
46arthropathy10.1
47esophagitis10.1
48glomerulonephritis10.1
49hypertrichosis10.1
50lung cancer10.1

Graphical network of the top 20 diseases related to Dermatomyositis:



Diseases related to dermatomyositis

Clinical Features for Dermatomyositis

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Sources:
46OMIM, 48Orphanet
See all sources

Clinical features from OMIM:

160750

Symptoms:

48 (show all 50)
  • cardiac rhythm disorder/arrhythmia
  • angor pectoris/myocardial infarction
  • vascularitis/vasculitides/arteritis
  • gangrena/necrosis
  • elocution disorders/dysarthria/dysphonia
  • eosinophils anomalies/hypereosinophilia
  • neoplasms/tumors
  • lung/bronchopulmonary neoplasm/tumor/carcinoma/cancer
  • breast neoplasm/tumor/carcinoma/cancer
  • ovary/fallopian tube neoplasm/tumor/carcinoma/cancer (excl. teratoma/germinoma)
  • gastrointestinal stromal tumor
  • lymphoma
  • fever/chilling
  • biological inflammatory syndrome/increased erythrocyte sedimentation rate/crp
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • myocardium anomalies/myocarditis
  • lung fibrosis
  • nails anomalies
  • hypotrichosis/atrichia/atrichiasis/scalp hairlessness
  • telangiectasic erythema/poikiloderma
  • pruritus/itching
  • follicular/erythematous/edematous papules/milium
  • chronic skin infection/ulcerations/ulcers/cancrum
  • dry/squaly skin/exfoliation
  • autoimmunity/autoimmune reaction/autoantibodies
  • abnormal emg/electromyogram/electropmyography
  • myalgia/muscular pain
  • muscle weakness/flaccidity
  • cutaneous edema
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • palpebral edema/periorbital edema
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • cough
  • interstitial/restrictive pneumopathy/restrictive respiratory syndrome
  • abnormal cry/voice/phonation disorder/nasal speech
  • pulmonary hypertension
  • feeding disorder/dysphagia/swallowing/sucking disorder/esophageal dyskinesia
  • telangiectasiae of the skin
  • skin photosensitivity
  • cellulitis/panniculitis/pseudocellulitis/inflammation of subcutaneous tissue
  • skin hypoplasia/aplasia/atrophy
  • asthenia/fatigue/weakness
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • periarticular tissue anomaly/extraarticular calcifications
  • arthritis/synovitis/synovial proliferation
  • articular/joint pain/arthralgia
  • hypotonia
  • acrocyanosis/raynaud's phenomenon/vasomotor disorders
  • repeat respiratory infections
  • anomalies of eyelids, eyelashes and lacrimal system

Drugs & Therapeutics for Dermatomyositis

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Dermatomyositis

Drug clinical trials:

Search ClinicalTrials for Dermatomyositis

Search NIH Clinical Center for Dermatomyositis

Search CenterWatch for Dermatomyositis

Genetic Tests for Dermatomyositis

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Anatomical Context for Dermatomyositis

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32MalaCards, 14FMA
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MalaCards organs/tissues related to Dermatomyositis:

32
Skin, Lung, Heart, Breast, T cells, Bone, Ovary, Testes, Thyroid, Monocytes, B cells, Endothelial, Tongue, Colon, Skeletal muscle, Prostate, Appendix, Brain, Liver, Myeloid

FMA organs/tissues related to Dermatomyositis:

14
Muscle or

Animal Models for Dermatomyositis or affiliated genes

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Publications for Dermatomyositis

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Sources:
50PubMed
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Articles related to Dermatomyositis:

(show top 50)    (show all 1016)
idTitleAuthorsYear
1
Limitations of a single-point evaluation of anti-MDA5 antibody, ferritin, and IL-18 in predicting the prognosis of interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis. (23250474)
2013
2
A case of interstitial lung disease associated with clinically amyopathic dermatomyositis: radiologic-pathologic correlation. (23047733)
2013
3
Myeloid related protein induces muscle derived inflammatory mediators in juvenile dermatomyositis. (24286299)
2013
4
The importance of including amyopathic dermatomyositis in the idiopathic inflammatory myositis spectrum. (23190767)
2013
5
Hemophagocytic lymphohistiocytosis complicated by central nervous system lesions in a patient with dermatomyositis: a case presentation and literature review. (22576792)
2013
6
Living-donor lobar lung transplantation for rapidly progressive interstitial pneumonia associated with clinically amyopathic dermatomyositis: report of a case. (22618987)
2013
7
MRI guided wire localization muscle biopsy in a child with juvenile dermatomyositis. (23566445)
2013
8
Influenza A H1N1/2009 vaccine in juvenile dermatomyositis: reduced immunogenicity in patients under immunosuppressive therapy. (22931582)
2012
9
Thrombotic microangiopathy and Purtscher-like retinopathy as a rare presentation of juvenile dermatomyositis. (22311994)
2012
10
Marked improvement of calcinosis in adult dermatomyositis with etidronate therapy. (21710176)
2012
11
Dramatic heliotrope dermatitis and uncommon pulmonary involvement in juvenile dermatomyositis. (22665709)
2012
12
Juvenile dermatomyositis: new insights and new treatment strategies. (22870494)
2012
13
Interferon and biologic signatures in dermatomyositis skin: specificity and heterogeneity across diseases. (22235269)
2012
14
Cardiac involvement in adult polymyositis or dermatomyositis: a systematic review. (22847365)
2012
15
Moderate incidence of prior rheumatoid arthritis in patients with polymyositis and dermatomyositis. (21523364)
2011
16
Pulmonary outcome in juvenile dermatomyositis: a case-control study. (20805295)
2011
17
Polymyositis and dermatomyositis: pathophysiology. (21444017)
2011
18
Results of the German ESPED-recording of new patients with juvenile dermatomyositis (JDM). (21509712)
2011
19
Relationship between cytomegalovirus reactivation and dermatomyositis. (21398206)
2011
20
Polymyositis, dermatomyositis and malignancy: a further intriguing link. (20026430)
2010
21
Dermatomyositis and small cell carcinoma of the bladder. (19826157)
2009
22
Damage extent and predictors in adult and juvenile dermatomyositis and polymyositis as determined with the myositis damage index. (19877055)
2009
23
Keratosis follicularis and dermatomyositis: is there a common pathogenesis? (19210696)
2009
24
Cytokine gene polymorphisms as risk and severity factors for juvenile dermatomyositis. (19035492)
2008
25
Preliminary validation and clinical meaning of the Cutaneous Assessment Tool in juvenile dermatomyositis. (18240194)
2008
26
Autoantibodies and their clinical significance in idiopathic inflammatory myopathies; polymyositis/dermatomyositis and related conditions]. (18174673)
2007
27
Step-up versus primary intensive approach to the treatment of interstitial pneumonia associated with dermatomyositis/polymyositis: a retrospective study. (17437167)
2007
28
Immunogenetic risk and protective factors for juvenile dermatomyositis in Caucasians. (17133612)
2006
29
Efficacy of probenecid for a patient with juvenile dermatomyositis complicated with calcinosis. (16881125)
2006
30
Disease course, frequency of relapses and survival of 73 patients with juvenile or adult dermatomyositis. (15789887)
2005
31
Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. (15880816)
2005
32
Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis. (14962966)
2004
33
Inflammatory myopathy in a patient with cutaneous findings of pityriasis rubra pilaris: a case of Wong's dermatomyositis. (14749988)
2004
34
Inflammatory disorders of muscle: progress in polymyositis, dermatomyositis and inclusion body myositis. (15367860)
2004
35
Update on juvenile dermatomyositis: new advances in understanding its etiopathogenesis. (12960487)
2003
36
Dermatomyositis and Polymyositis. (12895397)
2003
37
A survey on TCM treatment of polymyositis and dermatomyositis. (14535200)
2003
38
Infrapatellar hypertrichosis: an unusual cutaneous manifestation of juvenile dermatomyositis. (11994176)
2002
39
Difference in B cell activation between dermatomyositis and polymyositis: analysis of the expression of RP105 on peripheral blood B cells. (11709456)
2001
40
Dystrophic calcification in adult dermatomyositis: neuroimage. (11146618)
2000
41
Classical and amyopathic dermatomyositis seen at the National Skin Centre of Singapore: a 3-year retrospective review of their clinical characteristics and association with malignancy. (10895343)
2000
42
Paraneoplastic amyopathic dermatomyositis associated with breast cancer recurrence. (10534675)
1999
43
Amyopathic dermatomyositis complicated by interstitial pulmonary disease and pneumomediastinum. (9779873)
1998
44
A case of toxoplasmosis with dermatomyositis]. (8708414)
1995
45
Multicentric reticulohistiocytosis: presentation with the cutaneous features of dermatomyositis. (8162446)
1994
46
Early ultrastructural alterations in adult dermatomyositis. Capillary abnormalities precede other structural changes in muscle. (2614466)
1989
47
Prolonged spontaneous pneumomediastinum in adult dermatomyositis. (3662647)
1987
48
Dermatomyositis presenting with Ludwig's angina. (3468463)
1987
49
Cutaneous malakoplakia with dermatomyositis. (3570595)
1987
50
Systemic rheumatic disorders ("collagen disease") in childhood: lupus erythematosus, anaphylactoid purpura, dermatomyositis, and scleroderma. I. (6044284)
1967

Genetic Variations for Dermatomyositis

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Expression for genes affiliated with Dermatomyositis

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Dermatomyositis

Search GEO for disease gene expression data for Dermatomyositis.

Pathways for genes affiliated with Dermatomyositis

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Sources:
53Reactome, 29KEGG, 51QIAGEN
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Compounds for genes affiliated with Dermatomyositis

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Sources:
44Novoseek, 24HMDB, 11DrugBank, 49PharmGKB, 28IUPHAR, 59Tocris Bioscience
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Compounds related to Dermatomyositis according to GeneCards/GeneDecks:

(show all 32)
idCompoundScoreTop Affiliating Genes
1pyrophosphate44 2412.2CMAS, UBA2, KARS, HARS, GARS, PIK3C2A
2Adenosine triphosphate11 2412.2UBA2, KARS, HARS, GARS, PIK3C2A, CHKB
3phosphoric acid44 2412.2UBA2, KARS, HARS, GARS, IARS, CMAS
4Adenosine monophosphate11 2412.1UBA2, KARS, HARS, GARS, IARS
5polysaccharide4411.1SFTPD, ICAM1, CHKB, CMAS, VCAM1
6fluvastatin44 49 28 1114.1ICAM1, PIK3C2A, CHKB, VCAM1
7pravastatin44 49 28 11 2415.1VCAM1, CHKB, PIK3C2A, ICAM1
8alanine4411.0CCR7, KARS, GARS, PIK3C2A, CHKB, VCAM1
9atorvastatin44 49 28 11 2415.0VCAM1, CHKB, PIK3C2A, ICAM1
10prednisolone44 28 1113.0VCAM1, CHKB, PIK3C2A, SFTPD
11simvastatin44 49 59 28 11 2416.0ICAM1, PIK3C2A, CHKB, VCAM1
12lactate4411.0SFTPD, ICAM1, PIK3C2A, CHKB, VCAM1
13endotoxin4411.0SFTPD, ICAM1, CHKB, VCAM1
14aminoacyl-trna4411.0KARS, GARS, IARS
15budesonide44 49 1113.0SFTPD, ICAM1, VCAM1
16cerivastatin44 49 28 1114.0CHKB, PIK3C2A, ICAM1
17trna4410.9IARS, GARS, KARS
18fenofibrate44 49 1112.9VCAM1, PIK3C2A, ICAM1
19alpha-hydroxybutyrate4410.9PIK3C2A, CHKB
20thallium4410.9PIK3C2A, CHKB
21fibrinogen4410.9ICAM1, PIK3C2A, CHKB, VCAM1
22tetramethylpyrazine4410.8CHKB, ICAM1
23hydroxylysine4410.8SFTPD, CHKB
24sodium cromoglycate4410.8VCAM1, ICAM1
25dantrolene44 28 1112.8CHKB, PIK3C2A
26succinylcholine44 28 1112.7CHKB, PIK3C2A
278 methoxypsoralen4410.7VCAM1, ICAM1
28loratadine44 11 2412.6VCAM1, ICAM1
29anticodon4410.6GARS, KARS
30norepinephrine44 11 2412.6CCR7, CHKB
31gamma-hydroxybutyrate4410.5CHKB, PIK3C2A
32sele4410.3VCAM1, ICAM1

GO Terms for genes affiliated with Dermatomyositis

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16Gene Ontology
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Cellular components related to Dermatomyositis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytoplasmGO:00573710.9IFIT3, IFIH1, UBA2, KARS, HARS, GARS
2cytosolGO:00582910.9IFIT3, IFIH1, KARS, HARS, GARS, PIK3C2A
3ER to Golgi transport vesicle membraneGO:01250710.7STX17, HLA-DQA1, HLA-DQA2
4NuRD complexGO:01658110.6CHD4, CHD3
5MHC class II protein complexGO:04261310.3HLA-DQA1, HLA-DQA2

Biological processes related to Dermatomyositis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1tRNA aminoacylation for protein translationGO:00641810.8IARS, GARS, HARS, KARS
2interferon-gamma-mediated signaling pathwayGO:06033310.7ICAM1, HLA-DQA2, HLA-DQA1, VCAM1
3cytokine-mediated signaling pathwayGO:01922110.7IFIT3, ICAM1, HLA-DQA2, HLA-DQA1, VCAM1
4modulation by virus of host morphology or physiologyGO:01904810.7VCAM1, KARS, ICAM1, IFIH1
5diadenosine tetraphosphate biosynthetic processGO:01596610.5GARS, KARS
6membrane to membrane dockingGO:02261410.3ICAM1, VCAM1

Molecular functions related to Dermatomyositis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1ATP bindingGO:00552410.7IARS, IFIH1, UBA2, KARS, HARS, GARS
2MHC class II receptor activityGO:03239510.6HLA-DQA1, HLA-DQA2

Products for genes affiliated with Dermatomyositis

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Sources for Dermatomyositis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet