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Juvenile Dermatomyositis Sine Myositis

MedGen UID:
858244
•Concept ID:
C3898650
•
Disease or Syndrome
Synonym: Juvenile Amyopathic Dermatomyositis

Definition

A rare form of juvenile dermatomyositis that manifests with characteristic cutaneous findings for at least six months in the absence of any detectable muscle involvement. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVJuvenile Dermatomyositis Sine Myositis

Professional guidelines

PubMed

Comparison of the 2017 EULAR/ACR Criteria with Clinicoserologic Criteria for the Classification of Idiopathic Inflammatory Myopathies in Korean Patients.
Chung SW, Yoo IS, Kim J, Kang SW, Kwon M, Joung CI, Choi IA, Chang SH, Kang MI, Hong SJ, Lee YA
Yonsei Med J 2021 May;62(5):424-430. doi: 10.3349/ymj.2021.62.5.424. PMID: 33908213Free 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
Association of Dermatomyositis Sine Dermatitis With Anti-Nuclear Matrix Protein 2 Autoantibodies.
Inoue M, Tanboon J, Hirakawa S, Komaki H, Fukushima T, Awano H, Tajima T, Yamazaki K, Hayashi R, Mori T, Shibuya K, Yamanoi T, Yoshimura H, Ogawa T, Katayama A, Sugai F, Nakayama Y, Yamaguchi S, Hayashi S, Noguchi S, Tachimori H, Okiyama N, Fujimoto M, Nishino I
JAMA Neurol 2020 Jul 1;77(7):872-877. doi: 10.1001/jamaneurol.2020.0673. PMID: 32310254Free PMC Article
Dermatomyositis sine myositis: association with malignancy.
Whitmore SE, Watson R, Rosenshein NB, Provost TT
J Rheumatol 1996 Jan;23(1):101-5. PMID: 8838516
Dermatologic aspects of myositis.
Euwer RL, Sontheimer RD
Curr Opin Rheumatol 1994 Nov;6(6):583-9. doi: 10.1097/00002281-199411000-00006. PMID: 7865377
Amyopathic dermatomyositis: a review.
Euwer RL, Sontheimer RD
J Invest Dermatol 1993 Jan;100(1):124S-127S. doi: 10.1111/1523-1747.ep12356896. PMID: 8423381

Diagnosis

Association of Dermatomyositis Sine Dermatitis With Anti-Nuclear Matrix Protein 2 Autoantibodies.
Inoue M, Tanboon J, Hirakawa S, Komaki H, Fukushima T, Awano H, Tajima T, Yamazaki K, Hayashi R, Mori T, Shibuya K, Yamanoi T, Yoshimura H, Ogawa T, Katayama A, Sugai F, Nakayama Y, Yamaguchi S, Hayashi S, Noguchi S, Tachimori H, Okiyama N, Fujimoto M, Nishino I
JAMA Neurol 2020 Jul 1;77(7):872-877. doi: 10.1001/jamaneurol.2020.0673. PMID: 32310254Free PMC Article
Mixed connective tissue disease.
Gunnarsson R, Hetlevik SO, Lilleby V, Molberg Ø
Best Pract Res Clin Rheumatol 2016 Feb;30(1):95-111. Epub 2016 Apr 12 doi: 10.1016/j.berh.2016.03.002. PMID: 27421219
The clinical features, diagnosis and classification of dermatomyositis.
Iaccarino L, Ghirardello A, Bettio S, Zen M, Gatto M, Punzi L, Doria A
J Autoimmun 2014 Feb-Mar;48-49:122-7. Epub 2014 Jan 24 doi: 10.1016/j.jaut.2013.11.005. PMID: 24467910
Amyopathic dermatomyositis: a review.
Euwer RL, Sontheimer RD
J Invest Dermatol 1993 Jan;100(1):124S-127S. doi: 10.1111/1523-1747.ep12356896. PMID: 8423381
Amyopathic dermatomyositis (dermatomyositis siné myositis). Presentation of six new cases and review of the literature.
Euwer RL, Sontheimer RD
J Am Acad Dermatol 1991 Jun;24(6 Pt 1):959-66. PMID: 1869684

Therapy

Anti-NXP2 Antibody-positive Juvenile Dermatomyositis with Characteristic Fascial Thickening on Muscle Ultrasound and Improvement with Immunotherapy.
Fukushima K, Yoshida T, Yamazaki H, Takamatsu N, Nagai T, Osaki Y, Harada M, Nishino I, Okiyama N, Sugie K, Izumi Y
Intern Med 2024 Jun 15;63(12):1813-1817. Epub 2023 Nov 6 doi: 10.2169/internalmedicine.2720-23. PMID: 37926535Free PMC Article
An elusive case of dermatomyositis.
Ahsan T, Erum U
J Pak Med Assoc 2017 Nov;67(11):1756-1758. PMID: 29171576
Cutaneous dermatomyositis in the era of biologicals.
Wright NA, Vleugels RA, Callen JP
Semin Immunopathol 2016 Jan;38(1):113-21. Epub 2015 Nov 12 doi: 10.1007/s00281-015-0543-z. PMID: 26563285
Rheumatic syndromes: clues to occult neoplasia.
Naschitz JE, Rosner I, Rozenbaum M, Zuckerman E, Yeshurun D
Semin Arthritis Rheum 1999 Aug;29(1):43-55. doi: 10.1016/s0049-0172(99)80037-7. PMID: 10468414
Amyopathic dermatomyositis (dermatomyositis siné myositis). Presentation of six new cases and review of the literature.
Euwer RL, Sontheimer RD
J Am Acad Dermatol 1991 Jun;24(6 Pt 1):959-66. PMID: 1869684

Prognosis

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
Mixed connective tissue disease.
Gunnarsson R, Hetlevik SO, Lilleby V, Molberg Ø
Best Pract Res Clin Rheumatol 2016 Feb;30(1):95-111. Epub 2016 Apr 12 doi: 10.1016/j.berh.2016.03.002. PMID: 27421219
A systematic review of juvenile-onset clinically amyopathic dermatomyositis.
Gerami P, Walling HW, Lewis J, Doughty L, Sontheimer RD
Br J Dermatol 2007 Oct;157(4):637-44. Epub 2007 Jun 26 doi: 10.1111/j.1365-2133.2007.08055.x. PMID: 17596148
Dermatologic aspects of myositis.
Euwer RL, Sontheimer RD
Curr Opin Rheumatol 1994 Nov;6(6):583-9. doi: 10.1097/00002281-199411000-00006. PMID: 7865377
Amyopathic dermatomyositis (dermatomyositis siné myositis). Presentation of six new cases and review of the literature.
Euwer RL, Sontheimer RD
J Am Acad Dermatol 1991 Jun;24(6 Pt 1):959-66. PMID: 1869684

Clinical prediction guides

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
Anti-nuclear matrix protein 2 antibody-positive inflammatory myopathies represent extensive myositis without dermatomyositis-specific rash.
Ichimura Y, Konishi R, Shobo M, Inoue S, Okune M, Maeda A, Tanaka R, Kubota N, Matsumoto I, Ishii A, Tamaoka A, Shimbo A, Mori M, Morio T, Kishi T, Miyamae T, Tanboon J, Inoue M, Nishino I, Fujimoto M, Nomura T, Okiyama N
Rheumatology (Oxford) 2022 Mar 2;61(3):1222-1227. doi: 10.1093/rheumatology/keab518. PMID: 34152410
The significance of dermoscopy and trichoscopy in differentiation of erythroderma due to various dermatological disorders.
Sławińska M, Sokołowska-Wojdyło M, Sobjanek M, Golińska J, Nowicki RJ, Rudnicka L
J Eur Acad Dermatol Venereol 2021 Jan;35(1):230-240. Epub 2020 Dec 8 doi: 10.1111/jdv.16998. PMID: 33073390
A systematic review of juvenile-onset clinically amyopathic dermatomyositis.
Gerami P, Walling HW, Lewis J, Doughty L, Sontheimer RD
Br J Dermatol 2007 Oct;157(4):637-44. Epub 2007 Jun 26 doi: 10.1111/j.1365-2133.2007.08055.x. PMID: 17596148
A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies.
Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD
J Am Acad Dermatol 2006 Apr;54(4):597-613. Epub 2006 Jan 23 doi: 10.1016/j.jaad.2005.10.041. PMID: 16546580

Recent systematic reviews

A systematic review of juvenile-onset clinically amyopathic dermatomyositis.
Gerami P, Walling HW, Lewis J, Doughty L, Sontheimer RD
Br J Dermatol 2007 Oct;157(4):637-44. Epub 2007 Jun 26 doi: 10.1111/j.1365-2133.2007.08055.x. PMID: 17596148
A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies.
Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD
J Am Acad Dermatol 2006 Apr;54(4):597-613. Epub 2006 Jan 23 doi: 10.1016/j.jaad.2005.10.041. PMID: 16546580

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