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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 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
Consensus-based recommendations for the management of juvenile dermatomyositis.
Bellutti Enders F, Bader-Meunier B, Baildam E, Constantin T, Dolezalova P, Feldman BM, Lahdenne P, Magnusson B, Nistala K, Ozen S, Pilkington C, Ravelli A, Russo R, Uziel Y, van Brussel M, van der Net J, Vastert S, Wedderburn LR, Wulffraat N, McCann LJ, van Royen-Kerkhof A
Ann Rheum Dis 2017 Feb;76(2):329-340. Epub 2016 Aug 11 doi: 10.1136/annrheumdis-2016-209247. PMID: 27515057Free PMC Article

Recent clinical studies

Etiology

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. 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
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

Diagnosis

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
Dermatomyositis.
O'Connell KA, LaChance AH
N Engl J Med 2021 Jun 24;384(25):2437. doi: 10.1056/NEJMicm2033425. PMID: 34161709
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
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

Therapy

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
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
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

Prognosis

Cardiac Manifestations in Idiopathic Inflammatory Myopathies: An Overview.
Jayakumar D, Zhang R, Wasserman A, Ash J
Cardiol Rev 2019 May/Jun;27(3):131-137. doi: 10.1097/CRD.0000000000000241. PMID: 30585794
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
Risk of Malignancy in Dermatomyositis and Polymyositis.
Qiang JK, Kim WB, Baibergenova A, Alhusayen R
J Cutan Med Surg 2017 Mar/Apr;21(2):131-136. Epub 2016 Aug 20 doi: 10.1177/1203475416665601. PMID: 27534779
Amyopathic dermatomyositis.
Olsen NJ, Park JH, King LE Jr
Curr Rheumatol Rep 2001 Aug;3(4):346-51. doi: 10.1007/s11926-001-0039-9. PMID: 11470054
Dermatomyositis and malignancy.
Bernard P, Bonnetblanc JM
J Invest Dermatol 1993 Jan;100(1):128S-132S. doi: 10.1111/1523-1747.ep12356956. PMID: 8423382

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
Dermatomyositis: Muscle Pathology According to Antibody Subtypes.
Tanboon J, Inoue M, Saito Y, Tachimori H, Hayashi S, Noguchi S, Okiyama N, Fujimoto M, Nishino I
Neurology 2022 Feb 15;98(7):e739-e749. Epub 2021 Dec 6 doi: 10.1212/WNL.0000000000013176. PMID: 34873015Free PMC Article
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
JAK-inhibitors for dermatomyositis: A concise literature review.
Paudyal A, Zheng M, Lyu L, Thapa C, Gong S, Yang Y, Lyu X
Dermatol Ther 2021 May;34(3):e14939. Epub 2021 Mar 23 doi: 10.1111/dth.14939. PMID: 33713527

Recent systematic reviews

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
JAK-inhibitors for dermatomyositis: A concise literature review.
Paudyal A, Zheng M, Lyu L, Thapa C, Gong S, Yang Y, Lyu X
Dermatol Ther 2021 May;34(3):e14939. Epub 2021 Mar 23 doi: 10.1111/dth.14939. PMID: 33713527
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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