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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.
 
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

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
Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460

Recent clinical studies

Etiology

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
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
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
Li D, Tansley SL
Curr Rheumatol Rep 2019 Dec 11;21(12):74. doi: 10.1007/s11926-019-0871-4. PMID: 31828535Free PMC Article
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

O'Connell KA, LaChance AH
N Engl J Med 2021 Jun 24;384(25):2437. doi: 10.1056/NEJMicm2033425. PMID: 34161709
Leung AKC, Lam JM, Alobaida S, Leong KF, Wong AHC
Curr Pediatr Rev 2021;17(4):273-287. doi: 10.2174/1573396317666210426105045. PMID: 33902423
Li D, Tansley SL
Curr Rheumatol Rep 2019 Dec 11;21(12):74. doi: 10.1007/s11926-019-0871-4. PMID: 31828535Free PMC Article
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317
Bohan A, Peter JB
N Engl J Med 1975 Feb 13;292(7):344-7. doi: 10.1056/NEJM197502132920706. PMID: 1090839

Therapy

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
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
Leung AKC, Lam JM, Alobaida S, Leong KF, Wong AHC
Curr Pediatr Rev 2021;17(4):273-287. doi: 10.2174/1573396317666210426105045. PMID: 33902423
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317

Prognosis

Xu A, Ye Y, Fu Q, Lian X, Chen S, Guo Q, Lu LJ, Dai M, Lv X, Bao C
Rheumatology (Oxford) 2021 Jul 1;60(7):3343-3351. doi: 10.1093/rheumatology/keaa786. PMID: 33331866
Lian X, Zou J, Guo Q, Chen S, Lu L, Wang R, Zhou M, Fu Q, Ye Y, Bao C
Chest 2020 Oct;158(4):1535-1545. Epub 2020 May 16 doi: 10.1016/j.chest.2020.04.057. PMID: 32428508
Huber AM
Pediatr Clin North Am 2018 Aug;65(4):739-756. doi: 10.1016/j.pcl.2018.04.006. PMID: 30031496
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
Bernard P, Bonnetblanc JM
J Invest Dermatol 1993 Jan;100(1):128S-132S. doi: 10.1111/1523-1747.ep12356956. PMID: 8423382

Clinical prediction guides

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
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
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
Leung AKC, Lam JM, Alobaida S, Leong KF, Wong AHC
Curr Pediatr Rev 2021;17(4):273-287. doi: 10.2174/1573396317666210426105045. PMID: 33902423
Wu JQ, Lu MP, Reed AM
World J Pediatr 2020 Feb;16(1):31-43. Epub 2019 Sep 26 doi: 10.1007/s12519-019-00313-8. PMID: 31556011

Recent systematic reviews

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
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
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
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
Voet NB, van der Kooi EL, Riphagen II, Lindeman E, van Engelen BG, Geurts AC
Cochrane Database Syst Rev 2013 Jul 9;(7):CD003907. doi: 10.1002/14651858.CD003907.pub4. PMID: 23835682

Supplemental Content

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