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Dermatomyositis

MedGen UID:
8331
•Concept ID:
C0011633
•
Disease or Syndrome
Synonyms: Dermatopolymyositis; Polymyositis Dermatomyositis; Polymyositis-Dermatomyositis
SNOMED CT: Dermatomyositis (396230008); Wagner-Unverricht syndrome (396230008); Polymyositis with skin involvement (396230008); DM - Dermatomyositis (396230008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016367
Orphanet: ORPHA221

Definition

A rare idiopathic inflammatory myopathy (IIM) characterized by evocative skin lesions, muscle involvement with symmetrical proximal muscle weakness, and specific histological features. The clinical subtypes are defined by the presence of myositis-specific antibodies (anti-Mi2, anti-NXP2, anti-TIF1-γ, anti-MDA5, or anti-SAE antibodies) and are associated with specific clinical phenotypes and prognosis. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDermatomyositis
Follow this link to review classifications for Dermatomyositis in Orphanet.

Professional guidelines

PubMed

Dermatomyositis: Diagnosis and treatment.
Waldman R, DeWane ME, Lu J
J Am Acad Dermatol 2020 Feb;82(2):283-296. Epub 2019 Jul 4 doi: 10.1016/j.jaad.2019.05.105. PMID: 31279813
Current Classification and Management of Inflammatory Myopathies.
Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article
2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups.
Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, Visser M, Alfredsson L, Amato AA, Barohn RJ, Liang MH, Singh JA, Aggarwal R, Arnardottir S, Chinoy H, Cooper RG, Dankó K, Dimachkie MM, Feldman BM, Torre IG, Gordon P, Hayashi T, Katz JD, Kohsaka H, Lachenbruch PA, Lang BA, Li Y, Oddis CV, Olesinska M, Reed AM, Rutkowska-Sak L, Sanner H, Selva-O'Callaghan A, Song YW, Vencovsky J, Ytterberg SR, Miller FW, Rider LG; International Myositis Classification Criteria Project consortium, The Euromyositis register and The Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland)
Ann Rheum Dis 2017 Dec;76(12):1955-1964. Epub 2017 Oct 27 doi: 10.1136/annrheumdis-2017-211468. PMID: 29079590Free PMC Article

Recent clinical studies

Etiology

Juvenile dermatomyositis. Where are we now?
McCann LJ, Livermore P, Wilkinson MGL, Wedderburn LR
Clin Exp Rheumatol 2022 Feb;40(2):394-403. Epub 2022 Feb 7 doi: 10.55563/clinexprheumatol/56ilob. PMID: 35225221
Dermatomyositis: Diagnosis and treatment.
Waldman R, DeWane ME, Lu J
J Am Acad Dermatol 2020 Feb;82(2):283-296. Epub 2019 Jul 4 doi: 10.1016/j.jaad.2019.05.105. PMID: 31279813
Dermatomyositis: Clinical features and pathogenesis.
DeWane ME, Waldman R, Lu J
J Am Acad Dermatol 2020 Feb;82(2):267-281. Epub 2019 Jul 4 doi: 10.1016/j.jaad.2019.06.1309. PMID: 31279808
Juvenile Dermatomyositis-Clinical Phenotypes.
Li D, Tansley SL
Curr Rheumatol Rep 2019 Dec 11;21(12):74. doi: 10.1007/s11926-019-0871-4. PMID: 31828535Free PMC Article
Dermatomyositis.
Mahil S, Marks D, McCormack M, Rahman A
Br J Hosp Med (Lond) 2012 Feb;73(2):C18-22. doi: 10.12968/hmed.2012.73.sup2.c18. PMID: 22504710

Diagnosis

Dermatomyositis.
Callen JP
JAMA Dermatol 2023 Sep 1;159(9):1016. doi: 10.1001/jamadermatol.2023.1013. PMID: 37405743
Anti-MDA5 dermatomyositis: an update from bench to bedside.
Fuzzi E, Gatto M, Zen M, Franco C, Zanatta E, Ghirardello A, Doria A
Curr Opin Rheumatol 2022 Nov 1;34(6):365-373. Epub 2022 Sep 12 doi: 10.1097/BOR.0000000000000908. PMID: 36094462Free PMC Article
Paraneoplastic Dermatomyositis.
Orton C, Topham C, Madigan LM
Am J Med 2022 Aug;135(8):969-971. Epub 2022 Feb 24 doi: 10.1016/j.amjmed.2022.01.016. PMID: 35219689
Juvenile Dermatomyositis-Clinical Phenotypes.
Li D, Tansley SL
Curr Rheumatol Rep 2019 Dec 11;21(12):74. doi: 10.1007/s11926-019-0871-4. PMID: 31828535Free PMC Article
Dermatomyositis.
Chu LL, Rohekar G
CMAJ 2019 Mar 25;191(12):E340. doi: 10.1503/cmaj.180947. PMID: 30910882Free PMC Article

Therapy

Current myositis clinical trials and tribulations.
Saygin D, Werth V, Paik JJ, Park JK, Needham M, Lundberg IE, Christopher-Stine L
Ann Rheum Dis 2024 Jun 12;83(7):826-829. doi: 10.1136/ard-2023-224652. PMID: 38216318
Juvenile Dermatomyositis: Advances in Pathogenesis, Assessment, and Management.
Leung AKC, Lam JM, Alobaida S, Leong KF, Wong AHC
Curr Pediatr Rev 2021;17(4):273-287. doi: 10.2174/1573396317666210426105045. PMID: 33902423
Current diagnosis and treatment of polymyositis and dermatomyositis.
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
An overview of polymyositis and dermatomyositis.
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317
Mycophenolate in dermatology.
Liu V, Mackool BT
J Dermatolog Treat 2003 Dec;14(4):203-11. doi: 10.1080/09546630310016826. PMID: 14660264

Prognosis

Juvenile Idiopathic Inflammatory Myopathies.
Huber AM
Pediatr Clin North Am 2018 Aug;65(4):739-756. doi: 10.1016/j.pcl.2018.04.006. PMID: 30031496
Periorbital edema.
Jaeger T, Mourantchanian V, Gutermuth J, Hein R, Andres C, Ring J, Brockow K
Int J Dermatol 2014 Aug;53(8):937-9. Epub 2014 Apr 16 doi: 10.1111/ijd.12478. PMID: 24738945
Poikilodermatomyositis.
Perales-Martínez DE, Moncada B, Rolón-Padilla A, Chávez-López MA
Reumatol Clin 2012 Jul-Aug;8(4):227-8. Epub 2011 Dec 19 doi: 10.1016/j.reuma.2011.10.013. PMID: 22189157
Dermatomyositis.
Schaller JG
J Pediatr 1973 Oct;83(4):699-702. doi: 10.1016/s0022-3476(73)80252-5. PMID: 4581018
Juvenile dermatomyositis.
Hill RH, Wood WS
Can Med Assoc J 1970 Nov 21;103(11):1152-6. PMID: 4321324Free PMC Article

Clinical prediction guides

Trial of Intravenous Immune Globulin in Dermatomyositis.
Aggarwal R, Charles-Schoeman C, Schessl J, Bata-Csörgő Z, Dimachkie MM, Griger Z, Moiseev S, Oddis C, Schiopu E, Vencovský J, Beckmann I, Clodi E, Bugrova O, Dankó K, Ernste F, Goyal NA, Heuer M, Hudson M, Hussain YM, Karam C, Magnolo N, Nelson R, Pozur N, Prystupa L, Sárdy M, Valenzuela G, van der Kooi AJ, Vu T, Worm M, Levine T; ProDERM Trial Group
N Engl J Med 2022 Oct 6;387(14):1264-1278. doi: 10.1056/NEJMoa2117912. PMID: 36198179
Update on dermatomyositis.
Tanboon J, Nishino I
Curr Opin Neurol 2022 Oct 1;35(5):611-621. Epub 2022 Aug 4 doi: 10.1097/WCO.0000000000001091. PMID: 35942671
Juvenile Dermatomyositis: Advances in Pathogenesis, Assessment, and Management.
Leung AKC, Lam JM, Alobaida S, Leong KF, Wong AHC
Curr Pediatr Rev 2021;17(4):273-287. doi: 10.2174/1573396317666210426105045. PMID: 33902423
2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.
Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM, Oddis CV, Amato AA, Chinoy H, Cooper RG, Dastmalchi M, Fiorentino D, Isenberg D, Katz JD, Mammen A, de Visser M, Ytterberg SR, Lundberg IE, Chung L, Danko K, García-De la Torre I, Song YW, Villa L, Rinaldi M, Rockette H, Lachenbruch PA, Miller FW, Vencovsky J; International Myositis Assessment and Clinical Studies Group and the Paediatric Rheumatology International Trials Organisation
Ann Rheum Dis 2017 May;76(5):792-801. doi: 10.1136/annrheumdis-2017-211400. PMID: 28385805Free PMC Article
Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: Physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), physician global damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI).
Rider LG, Werth VP, Huber AM, Alexanderson H, Rao AP, Ruperto N, Herbelin L, Barohn R, Isenberg D, Miller FW
Arthritis Care Res (Hoboken) 2011 Nov;63 Suppl 11(0 11):S118-57. doi: 10.1002/acr.20532. PMID: 22588740Free PMC Article

Recent systematic reviews

Eosinophils in Traditionally Noneosinophil-Rich Dermatoses.
Sanchez I, Ibraheim MK, Lee BA, Kraus CN, Elsensohn A
Am J Dermatopathol 2023 Dec 1;45(12):820-821. doi: 10.1097/DAD.0000000000002550. PMID: 37883982
Use of Janus kinase inhibitors in dermatomyositis: a systematic literature review.
Paik JJ, Lubin G, Gromatzky A, Mudd PN Jr, Ponda MP, Christopher-Stine L
Clin Exp Rheumatol 2023 Mar;41(2):348-358. Epub 2022 Jun 28 doi: 10.55563/clinexprheumatol/hxin6o. PMID: 35766013Free PMC Article
Management of MDA-5 antibody positive clinically amyopathic dermatomyositis associated interstitial lung disease: A systematic review.
McPherson M, Economidou S, Liampas A, Zis P, Parperis K
Semin Arthritis Rheum 2022 Apr;53:151959. Epub 2022 Jan 31 doi: 10.1016/j.semarthrit.2022.151959. PMID: 35134633
Treatment of calcinosis cutis in systemic sclerosis and dermatomyositis: A review of the literature.
Traineau H, Aggarwal R, Monfort JB, Senet P, Oddis CV, Chizzolini C, Barbaud A, Francès C, Arnaud L, Chasset F
J Am Acad Dermatol 2020 Feb;82(2):317-325. Epub 2019 Jul 11 doi: 10.1016/j.jaad.2019.07.006. PMID: 31302187
Strength training and aerobic exercise training for muscle disease.
Voet NB, van der Kooi EL, van Engelen BG, Geurts AC
Cochrane Database Syst Rev 2019 Dec 6;12(12):CD003907. doi: 10.1002/14651858.CD003907.pub5. PMID: 31808555Free PMC Article

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