Itchy Facial Rash

  • Yan Ling Kong
  • Emily Yiping Gan


  • Juvenile dermatomyositis (JDM) is a multisystemic disorder affecting children younger than 18 years old

  • Patients frequently present with skin and muscle involvement, but other organs such as the lungs, esophagus and heart may also be affected

  • Dermatomyositis is histologically indistinguishable from lupus erythematosus, and characterized by the presence of interface dermatitis, thickened basement membrane, perivascular lymphocytic infiltrate and increased mucin in the dermis

  • Direct immunofluorescence is often negative in dermatomyositis

  • Myositis-specific autoantibodies (MSA) are associated with a variety of clinical phenotypes in dermatomyositis

  • The most common MSA present in JDM are the anti-p155/140 autoantibodies

  • Methotrexate is the most common steroid-sparing oral immunosuppressant used in the treatment of juvenile dermatomyositis


Juvenile dermatomyositis Direct immunofluorescence Myositis-specific autoantibodies 


  1. 1.
    Orione M, Silva C, Sallum A, Campos L, Omori C, Braga A, Farhat S. Risk factors for juvenile dermatomyositis: exposure to tobacco and air pollutants during pregnancy. Arthritis Care Res. 2014;66(10):1571–5.CrossRefGoogle Scholar
  2. 2.
    Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol. 2006;24(5):363–73.CrossRefGoogle Scholar
  3. 3.
    Callen JP. Cutaneous manifestations of dermatomyositis and their management. Curr Rheumatol Rep. 2010;12(3):192–7.CrossRefGoogle Scholar
  4. 4.
    Singh S, Suri D, Aulakh R, Gupta A, Rawat A, Kumar RM. Mortality in children with juvenile dermatomyositis: two decades of experience from a single tertiary care centre in North India. Clin Rheumatol. 2014;33(11):1675–9.CrossRefGoogle Scholar
  5. 5.
    Ramanan AV, Feldman BM. Clinical features and outcomes of juvenile dermatomyositis and other childhood onset myositis syndromes. Rheum Dis Clin N Am. 2002;28(4):833–57.CrossRefGoogle Scholar
  6. 6.
    Walling HW, Gerami P, Sontheimer RD. Juvenile-onset clinically amyopathic dermatomyositis: an overview of recent progress in diagnosis and management. Paediatr Drugs. 2010;12(1):23–34.CrossRefGoogle Scholar
  7. 7.
    Reed AM, Pachman L, Ober C. Molecular genetic studies of major histocompatibility complex genes in children with juvenile dermatomyositis: increased risk associated with HLA-DQA1 ∗0501. Hum Immunol. 1991;32(4):235–40.CrossRefGoogle Scholar
  8. 8.
    Stockton D, Doherty VR, Brewster DH. Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish population-based cohort study. Br J Cancer. 2001;85(1):41–5.CrossRefGoogle Scholar
  9. 9.
    Burg G, Kempf W, Kutzner H, Feit J, Karai L. Atlas of dermatopathology. New York: Taylor & Francis Inc.; 2015.Google Scholar
  10. 10.
    Gunawardena H, Betteridge ZE, McHugh NJ. Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression. Rheumatology (Oxford). 2009;48(6):607–12.CrossRefGoogle Scholar
  11. 11.
    Kobayashi N, Takezaki S, Kobayashi I, Iwata N, Mori M, Nagai K, Nakano N, Miyoshi M, Kinjo N, Murata T, Masunaga K, Umebayashi H, Imagawa T, Agematsu K, Sato S, Kuwana M, Yamada M, Takei S, Yokota S, Koike K, Ariga T. Clinical and laboratory features of fatal rapidly progressive interstitial lung disease associated with juvenile dermatomyositis. Rheumatology (Oxford). 2015;54(5):784–91.CrossRefGoogle Scholar
  12. 12.
    Laskin BL, Choyke P, Keenan GF, Miller FW, Rider LG. Novel gastrointestinal tract manifestations in juvenile dermatomyositis. J Pediatr. 1999;135(3):371–4.CrossRefGoogle Scholar
  13. 13.
    Crowe W, Bove K, Levinson J, Hilton P. Clinical and pathogenetic implications of histopathology in childhood polydermatomyositis. Arthritis Rheum. 1982;25(2):126–39.CrossRefGoogle Scholar
  14. 14.
    Ramanan AV, Campbell-Webster N, Ota S, Parker S, Tran D, Tyrrell PN, Cameron B, Spiegel L, Schneider R, Laxer RM, Silverman ED, Feldman BM. The effectiveness of treating juvenile dermatomyositis with methotrexate and aggressively tapered corticosteroids. Arthritis Rheum. 2005;52(11):3570–8.CrossRefGoogle Scholar
  15. 15.
    Zieglschmid-Adams ME, Pandya AG, Cohen SB, Sontheimer RD. Treatment of dermatomyositis with methotrexate. J Am Acad Dermatol. 1995;32(5 Pt 1):754–7.CrossRefGoogle Scholar
  16. 16.
    Al-Mayouf SM, Laxer RM, Schneider R, Silverman ED, Feldman BM. Intravenous immunoglobulin therapy for juvenile dermatomyositis: efficacy and safety. J Rheumatol. 2000;27(10):2498–503.PubMedGoogle Scholar
  17. 17.
    Stringer E, Feldman BM. Advances in the treatment of juvenile dermatomyositis. Curr Opin Rheumatol. 2006;18(5):503–6.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Yan Ling Kong
    • 1
  • Emily Yiping Gan
    • 1
    • 2
  1. 1.National Skin CentreSingaporeSingapore
  2. 2.Dermatology ServiceKK Women’s and Children’s HospitalSingaporeSingapore

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