← Back to guidelines
Cardiology317 papers

Neonatal dermatomyositis

Last edited: 4/13/2026

Overview

Juvenile dermatomyositis (JDM) is a rare inflammatory myopathy primarily affecting children, characterized by muscle weakness and characteristic skin manifestations such as heliotrope rash and Gottron papules. 12323

Diagnosis

  • Clinical Criteria: Symmetrical muscle weakness, particularly in proximal muscles, and characteristic skin rashes. 1223
  • Laboratory Tests: Elevated muscle enzymes (CK, aldolase), ANA positivity, and myositis-specific antibodies (e.g., anti-MDA5). 121218
  • Imaging: MRI can show characteristic muscle inflammation but does not correlate with clinical inactive disease criteria. 8
  • Muscle Biopsy: Often required for definitive diagnosis, showing inflammatory changes. 9
  • Management

  • First-Line Treatments:
  • - Corticosteroids: High-dose oral or intravenous glucocorticoids (e.g., prednisone). 1213 - Immunosuppressants: Methotrexate, intravenous immunoglobulin (IVIG), or calcineurin inhibitors (e.g., tacrolimus). 13413
  • Adjunctive Treatments:
  • - Janus Kinase Inhibitors (JAKi): For refractory cases, e.g., tofacitinib. 3 - Biologic DMARDs: For refractory moderate JDM, such as TNF inhibitors or other biologics. 4 - Hydroxychloroquine: Considered for incomplete response to corticosteroids. 48

    Special Populations

  • Pediatrics: Management closely follows adult guidelines but with careful monitoring of growth and development. 723
  • Comorbidities: Special attention to complications like calcinosis, MAS, and malignancy associations. 102136
  • Key Recommendations

  • Targeted Therapy: Develop treatment plans targeting specific disease activity states using validated indices like JDMAI. (Evidence: Moderate) 5
  • Biologic DMARDs for Refractory Cases: Consider biologic DMARDs for patients with refractory moderate JDM who do not respond to conventional therapy. (Evidence: Moderate) 4
  • Monitoring and Response Criteria: Utilize validated response criteria (e.g., ACR/EULAR 2016 criteria) to assess treatment efficacy. (Evidence: Moderate) 25
  • Glucocorticoid Tapering: Base tapering on clinical and MRI criteria, recognizing MRI may not fully correlate with clinical inactive disease. (Evidence: Expert opinion) 8
  • Environmental Considerations: Consider environmental factors like UV exposure in managing disease severity. (Evidence: Moderate) 16
  • References

    1 Ravelli A, Rosina S, MacMahon JM, Baird T, Rebollo-Giménez AI, Hinze C et al.. Treating juvenile dermatomyositis to target: Paediatric Rheumatology European Society/Childhood Arthritis and Rheumatology Research Alliance-endorsed recommendations from an international task force. Annals of the rheumatic diseases 2025. link 2 Perfetto J, Lewandowski LB, Wahezi DM, Ogega V, Ahimbisibwe J, Webb K et al.. Exploring the clinical profiles and management of juvenile dermatomyositis in Africa: a survey of African rheumatology care providers. Pediatric rheumatology online journal 2025. link 3 Sherman MA, Nicolai R, Datyner EK, Rosina S, Hamilton A, Ardalan K et al.. Approach to Janus kinase inhibition for juvenile dermatomyositis among CARRA and PReS providers. Rheumatology (Oxford, England) 2025. link 4 Tarvin SE, Sherman MA, Kim H, Balmuri N, Brown AG, Chow A et al.. Childhood Arthritis and Rheumatology Research Alliance Biologic Disease-Modifying Antirheumatic Drug Consensus Treatment Plans for Refractory Moderately Severe Juvenile Dermatomyositis. Arthritis care & research 2024. link 5 Rosina S, Consolaro A, Pistorio A, Rebollo-Giménez A, Bracaglia C, Dolezalova P et al.. Defining criteria for disease activity states in juvenile dermatomyositis based on the Juvenile Dermatomyositis Activity Index. RMD open 2024. link 6 Sherman MA, Kim H, Banschbach K, Brown A, Gewanter HL, Lang B et al.. Treatment escalation patterns to start biologics in refractory moderate juvenile dermatomyositis among members of the Childhood Arthritis and Rheumatology Research Alliance. Pediatric rheumatology online journal 2023. link 7 Neely J, Ardalan K, Huber A, Kim S. Baseline characteristics of children with juvenile dermatomyositis enrolled in the first year of the new Childhood Arthritis and Rheumatology Research Alliance registry. Pediatric rheumatology online journal 2022. link 8 Gargh K, Al-Abadi E, Low S, Harrison K, Coles W, Davis P et al.. Juvenile Dermatomyositis Magnetic Resonance Imaging Score (JIS) does not correlate with criteria for clinically inactive disease: a single-centre retrospective evaluation. Rheumatology international 2022. link 9 Ahmed S, Concha JSS, Chakka S, Krain RL, Zamalin D, Foulke G et al.. Diagnosing muscle disease in a cohort of classic dermatomyositis patients seen at a rheumatologic dermatology outpatient clinic. Journal of the American Academy of Dermatology 2022. link 10 Stewart JA, Price T, Moser S, Mullikin D, Bryan A. Progressive, refractory macrophage activation syndrome as the initial presentation of anti-MDA5 antibody positive juvenile dermatomyositis: a case report and literature review. Pediatric rheumatology online journal 2022. link 11 Liu K, Tomlinson G, Reed AM, Huber AM, Saarela O, Bout-Tabaku SM et al.. Pilot Study of the Juvenile Dermatomyositis Consensus Treatment Plans: A CARRA Registry Study. The Journal of rheumatology 2021. link 12 Sag E, Demir S, Bilginer Y, Talim B, Haliloglu G, Ozen S. Validation of the EULAR/ACR 2017 idiopathic inflammatory myopathy classification criteria in juvenile dermatomyositis patients. Clinical and experimental rheumatology 2021. link 13 Kobayashi I, Akioka S, Kobayashi N, Iwata N, Takezaki S, Nakaseko H et al.. Clinical practice guidance for juvenile dermatomyositis (JDM) 2018-Update. Modern rheumatology 2020. link 14 Concha JSS, Pena S, Gaffney RG, Patel B, Tarazi M, Kushner CJ et al.. Developing classification criteria for skin-predominant dermatomyositis: the Delphi process. The British journal of dermatology 2020. link 15 Kohsaka H, Mimori T, Kanda T, Shimizu J, Sunada Y, Fujimoto M et al.. Treatment consensus for management of polymyositis and dermatomyositis among rheumatologists, neurologists and dermatologists. Modern rheumatology 2019. link 16 Neely J, Long CS, Sturrock H, Kim S. Association of Short-Term Ultraviolet Radiation Exposure and Disease Severity in Juvenile Dermatomyositis: Results From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. Arthritis care & research 2019. link 17 Concha JSS, Tarazi M, Kushner CJ, Gaffney RG, Werth VP. The diagnosis and classification of amyopathic dermatomyositis: a historical review and assessment of existing criteria. The British journal of dermatology 2019. link 18 Patel B, Khan N, Werth VP. Applicability of EULAR/ACR classification criteria for dermatomyositis to amyopathic disease. Journal of the American Academy of Dermatology 2018. link 19 Varnier GC, Rosina S, Ferrari C, Pistorio A, Consolaro A, Bovis F et al.. Development and Testing of a Hybrid Measure of Muscle Strength in Juvenile Dermatomyositis for Use in Routine Care. Arthritis care & research 2018. link 20 Takashima R, Takamatsu K, Shinkawa Y, Yagita M, Fukui M, Fujita M. Dermatomyositis Associated with Lung Neuroendocrine Carcinoma. Internal medicine (Tokyo, Japan) 2017. link 21 Orandi AB, Baszis KW, Dharnidharka VR, Huber AM, Hoeltzel MF. Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA). Pediatric rheumatology online journal 2017. link 22 Huber AM, Kim S, Reed AM, Carrasco R, Feldman BM, Hong SD et al.. Childhood Arthritis and Rheumatology Research Alliance Consensus Clinical Treatment Plans for Juvenile Dermatomyositis with Persistent Skin Rash. The Journal of rheumatology 2017. link 23 Barut K, Aydin PO, Adrovic A, Sahin S, Kasapcopur O. Juvenile dermatomyositis: a tertiary center experience. Clinical rheumatology 2017. link 24 Aggarwal R, Rider LG, Ruperto N, Bayat N, Erman B, Feldman BM et al.. 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. Arthritis & rheumatology (Hoboken, N.J.) 2017. link 25 Rider LG, Aggarwal R, Pistorio A, Bayat N, Erman B, Feldman BM et al.. 2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Arthritis & rheumatology (Hoboken, N.J.) 2017. link 26 Dulin JD, Coyne PJ, Bohm NM, Adler M. Fosaprepitant for the Management of Refractory Pain in a Patient with Cancer-Related Dermatomyositis. Journal of palliative medicine 2017. link 27 Sunderkötter C, Nast A, Worm M, Dengler R, Dörner T, Ganter H et al.. Guidelines on dermatomyositis--excerpt from the interdisciplinary S2k guidelines on myositis syndromes by the German Society of Neurology. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG 2016. link 28 Macía-Villa CC, Guillén-Astete CA, Larena-Grijalba C, Zea-Mendonza A. Fatal outcome in a case of dermatomyositis and Hamman-Rich syndrome. Acta reumatologica portuguesa 2014. link 29 Robinson AB, Hoeltzel MF, Wahezi DM, Becker ML, Kessler EA, Schmeling H et al.. Clinical characteristics of children with juvenile dermatomyositis: the Childhood Arthritis and Rheumatology Research Alliance Registry. Arthritis care & research 2014. link 30 Prasad S, Misra R, Agarwal V, Lawrence A, Aggarwal A. Juvenile dermatomyositis at a tertiary care hospital: is there any change in the last decade?. International journal of rheumatic diseases 2013. link 31 Gowdie PJ, Allen RC, Kornberg AJ, Akikusa JD. Clinical features and disease course of patients with juvenile dermatomyositis. International journal of rheumatic diseases 2013. link 32 Lazarevic D, Pistorio A, Palmisani E, Miettunen P, Ravelli A, Pilkington C et al.. The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis. Annals of the rheumatic diseases 2013. link 33 Nickavar A, Mehr Azma M. Nephrotic syndrome and juvenile dermatomyositis. Rheumatology international 2012. link 34 Callen JP. Cutaneous manifestations of dermatomyositis and their management. Current rheumatology reports 2010. link 35 Ruperto N, Pistorio A, Ravelli A, Rider LG, Pilkington C, Oliveira S et al.. The Paediatric Rheumatology International Trials Organisation provisional criteria for the evaluation of response to therapy in juvenile dermatomyositis. Arthritis care & research 2010. link 36 Tang MM, Thevarajah S. Paraneoplastic Dermatomyositis: A 12-year Retrospective Review in the Department of Dermatology Hospital Kuala Lumpur. The Medical journal of Malaysia 2010. link 37 Lemos EM, Santoro PP, Tavares RA, Garcia RID, Furia CLB. Oropharyngeal dysphagia in dermatomyosites: case report and literature review. Brazilian journal of otorhinolaryngology 2008. link30157-9) 38 Williams DS. Dermatomyositis. Journal of insurance medicine (New York, N.Y.) 2007. link 39 Klein RQ, Teal V, Taylor L, Troxel AB, Werth VP. Number, characteristics, and classification of patients with dermatomyositis seen by dermatology and rheumatology departments at a large tertiary medical center. Journal of the American Academy of Dermatology 2007. link 40 Wargula JC, Lovell DJ, Passo MH, Bove KE, Santangelo JD, Levinson JE. What more can we learn from muscle histopathology in children with dermatomyositis/polymyositis?. Clinical and experimental rheumatology 2006. link 41 Reed AM, Mason T. Recent advances in juvenile dermatomyositis. Current rheumatology reports 2005. link 42 Parulkar BG, Isen J, Trifilio D. Obstructive anuria and dermatomyositis: a unique association. Urology 1997. link00200-8) 43 Huang JL. Juvenile dermatomyositis associated with partial lipodystrophy. The British journal of clinical practice 1996. link 44 Yahata S, Endo T, Kawaguchi N, Aoki S, Katsuta Y, Hayakawa H. Dermatomyositis-polymyositis and malignancy. Is there a direct relation?. Nihon Ika Daigaku zasshi 1993. link 45 Hay EM, Makris M, Winfield J, Winfield DA. Evans' syndrome associated with dermatomyositis. Annals of the rheumatic diseases 1990. link 46 Cicuttini FM, Fraser KJ. Recurrent pneumomediastinum in adult dermatomyositis. The Journal of rheumatology 1989. link 47 Caro I. Dermatomyositis as a systemic disease. The Medical clinics of North America 1989. link30627-7) 48 Olson NY, Lindsley CB. Adjunctive use of hydroxychloroquine in childhood dermatomyositis. The Journal of rheumatology 1989. link 49 Cooper C, Fairris G, Cotton DW, Steart P, Barth JH. Dermatomyositis associated with idiopathic thrombocytopenia. Dermatologica 1986. link 50 Muelleman PJ, Perry HO. A skin marker for geriatric cancer. Geriatrics 1986. link 51 Pachman LM, Maryjowski MC. Juvenile dermatomyositis and polymyositis. Clinics in rheumatic diseases 1984. link 52 Simpson NB, Golding JR. Dermatomyositis induced by penicillamine. Acta dermato-venereologica 1979. link 53 Goldstein J. Dermatomyositis complicated by acute granulocytic leukeumia. Southern medical journal 1978. link 54 Fernandes L, Swinson DR, Hamilton EB. Dermatomyositis complicating penicillamine treatment. Annals of the rheumatic diseases 1977. link 55 Marks SH, McShane DJ, Mitchell DM. Dermatomyositis following rhabdomyolysis. The Journal of rheumatology 1976. link

    Original source

    1. [1]
    2. [2]
      Exploring the clinical profiles and management of juvenile dermatomyositis in Africa: a survey of African rheumatology care providers.Perfetto J, Lewandowski LB, Wahezi DM, Ogega V, Ahimbisibwe J, Webb K et al. Pediatric rheumatology online journal (2025)
    3. [3]
      Approach to Janus kinase inhibition for juvenile dermatomyositis among CARRA and PReS providers.Sherman MA, Nicolai R, Datyner EK, Rosina S, Hamilton A, Ardalan K et al. Rheumatology (Oxford, England) (2025)
    4. [4]
    5. [5]
      Defining criteria for disease activity states in juvenile dermatomyositis based on the Juvenile Dermatomyositis Activity Index.Rosina S, Consolaro A, Pistorio A, Rebollo-Giménez A, Bracaglia C, Dolezalova P et al. RMD open (2024)
    6. [6]
      Treatment escalation patterns to start biologics in refractory moderate juvenile dermatomyositis among members of the Childhood Arthritis and Rheumatology Research Alliance.Sherman MA, Kim H, Banschbach K, Brown A, Gewanter HL, Lang B et al. Pediatric rheumatology online journal (2023)
    7. [7]
    8. [8]
    9. [9]
      Diagnosing muscle disease in a cohort of classic dermatomyositis patients seen at a rheumatologic dermatology outpatient clinic.Ahmed S, Concha JSS, Chakka S, Krain RL, Zamalin D, Foulke G et al. Journal of the American Academy of Dermatology (2022)
    10. [10]
    11. [11]
      Pilot Study of the Juvenile Dermatomyositis Consensus Treatment Plans: A CARRA Registry Study.Liu K, Tomlinson G, Reed AM, Huber AM, Saarela O, Bout-Tabaku SM et al. The Journal of rheumatology (2021)
    12. [12]
      Validation of the EULAR/ACR 2017 idiopathic inflammatory myopathy classification criteria in juvenile dermatomyositis patients.Sag E, Demir S, Bilginer Y, Talim B, Haliloglu G, Ozen S Clinical and experimental rheumatology (2021)
    13. [13]
      Clinical practice guidance for juvenile dermatomyositis (JDM) 2018-Update.Kobayashi I, Akioka S, Kobayashi N, Iwata N, Takezaki S, Nakaseko H et al. Modern rheumatology (2020)
    14. [14]
      Developing classification criteria for skin-predominant dermatomyositis: the Delphi process.Concha JSS, Pena S, Gaffney RG, Patel B, Tarazi M, Kushner CJ et al. The British journal of dermatology (2020)
    15. [15]
      Treatment consensus for management of polymyositis and dermatomyositis among rheumatologists, neurologists and dermatologists.Kohsaka H, Mimori T, Kanda T, Shimizu J, Sunada Y, Fujimoto M et al. Modern rheumatology (2019)
    16. [16]
    17. [17]
      The diagnosis and classification of amyopathic dermatomyositis: a historical review and assessment of existing criteria.Concha JSS, Tarazi M, Kushner CJ, Gaffney RG, Werth VP The British journal of dermatology (2019)
    18. [18]
      Applicability of EULAR/ACR classification criteria for dermatomyositis to amyopathic disease.Patel B, Khan N, Werth VP Journal of the American Academy of Dermatology (2018)
    19. [19]
      Development and Testing of a Hybrid Measure of Muscle Strength in Juvenile Dermatomyositis for Use in Routine Care.Varnier GC, Rosina S, Ferrari C, Pistorio A, Consolaro A, Bovis F et al. Arthritis care & research (2018)
    20. [20]
      Dermatomyositis Associated with Lung Neuroendocrine Carcinoma.Takashima R, Takamatsu K, Shinkawa Y, Yagita M, Fukui M, Fujita M Internal medicine (Tokyo, Japan) (2017)
    21. [21]
    22. [22]
      Childhood Arthritis and Rheumatology Research Alliance Consensus Clinical Treatment Plans for Juvenile Dermatomyositis with Persistent Skin Rash.Huber AM, Kim S, Reed AM, Carrasco R, Feldman BM, Hong SD et al. The Journal of rheumatology (2017)
    23. [23]
      Juvenile dermatomyositis: a tertiary center experience.Barut K, Aydin PO, Adrovic A, Sahin S, Kasapcopur O Clinical rheumatology (2017)
    24. [24]
    25. [25]
    26. [26]
      Fosaprepitant for the Management of Refractory Pain in a Patient with Cancer-Related Dermatomyositis.Dulin JD, Coyne PJ, Bohm NM, Adler M Journal of palliative medicine (2017)
    27. [27]
      Guidelines on dermatomyositis--excerpt from the interdisciplinary S2k guidelines on myositis syndromes by the German Society of Neurology.Sunderkötter C, Nast A, Worm M, Dengler R, Dörner T, Ganter H et al. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG (2016)
    28. [28]
      Fatal outcome in a case of dermatomyositis and Hamman-Rich syndrome.Macía-Villa CC, Guillén-Astete CA, Larena-Grijalba C, Zea-Mendonza A Acta reumatologica portuguesa (2014)
    29. [29]
      Clinical characteristics of children with juvenile dermatomyositis: the Childhood Arthritis and Rheumatology Research Alliance Registry.Robinson AB, Hoeltzel MF, Wahezi DM, Becker ML, Kessler EA, Schmeling H et al. Arthritis care & research (2014)
    30. [30]
      Juvenile dermatomyositis at a tertiary care hospital: is there any change in the last decade?Prasad S, Misra R, Agarwal V, Lawrence A, Aggarwal A International journal of rheumatic diseases (2013)
    31. [31]
      Clinical features and disease course of patients with juvenile dermatomyositis.Gowdie PJ, Allen RC, Kornberg AJ, Akikusa JD International journal of rheumatic diseases (2013)
    32. [32]
      The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis.Lazarevic D, Pistorio A, Palmisani E, Miettunen P, Ravelli A, Pilkington C et al. Annals of the rheumatic diseases (2013)
    33. [33]
      Nephrotic syndrome and juvenile dermatomyositis.Nickavar A, Mehr Azma M Rheumatology international (2012)
    34. [34]
      Cutaneous manifestations of dermatomyositis and their management.Callen JP Current rheumatology reports (2010)
    35. [35]
      The Paediatric Rheumatology International Trials Organisation provisional criteria for the evaluation of response to therapy in juvenile dermatomyositis.Ruperto N, Pistorio A, Ravelli A, Rider LG, Pilkington C, Oliveira S et al. Arthritis care & research (2010)
    36. [36]
    37. [37]
      Oropharyngeal dysphagia in dermatomyosites: case report and literature review.Lemos EM, Santoro PP, Tavares RA, Garcia RID, Furia CLB Brazilian journal of otorhinolaryngology (2008)
    38. [38]
      Dermatomyositis.Williams DS Journal of insurance medicine (New York, N.Y.) (2007)
    39. [39]
    40. [40]
      What more can we learn from muscle histopathology in children with dermatomyositis/polymyositis?Wargula JC, Lovell DJ, Passo MH, Bove KE, Santangelo JD, Levinson JE Clinical and experimental rheumatology (2006)
    41. [41]
      Recent advances in juvenile dermatomyositis.Reed AM, Mason T Current rheumatology reports (2005)
    42. [42]
      Obstructive anuria and dermatomyositis: a unique association.Parulkar BG, Isen J, Trifilio D Urology (1997)
    43. [43]
      Juvenile dermatomyositis associated with partial lipodystrophy.Huang JL The British journal of clinical practice (1996)
    44. [44]
      Dermatomyositis-polymyositis and malignancy. Is there a direct relation?Yahata S, Endo T, Kawaguchi N, Aoki S, Katsuta Y, Hayakawa H Nihon Ika Daigaku zasshi (1993)
    45. [45]
      Evans' syndrome associated with dermatomyositis.Hay EM, Makris M, Winfield J, Winfield DA Annals of the rheumatic diseases (1990)
    46. [46]
      Recurrent pneumomediastinum in adult dermatomyositis.Cicuttini FM, Fraser KJ The Journal of rheumatology (1989)
    47. [47]
      Dermatomyositis as a systemic disease.Caro I The Medical clinics of North America (1989)
    48. [48]
      Adjunctive use of hydroxychloroquine in childhood dermatomyositis.Olson NY, Lindsley CB The Journal of rheumatology (1989)
    49. [49]
      Dermatomyositis associated with idiopathic thrombocytopenia.Cooper C, Fairris G, Cotton DW, Steart P, Barth JH Dermatologica (1986)
    50. [50]
      A skin marker for geriatric cancer.Muelleman PJ, Perry HO Geriatrics (1986)
    51. [51]
      Juvenile dermatomyositis and polymyositis.Pachman LM, Maryjowski MC Clinics in rheumatic diseases (1984)
    52. [52]
      Dermatomyositis induced by penicillamine.Simpson NB, Golding JR Acta dermato-venereologica (1979)
    53. [53]
      Dermatomyositis complicated by acute granulocytic leukeumia.Goldstein J Southern medical journal (1978)
    54. [54]
      Dermatomyositis complicating penicillamine treatment.Fernandes L, Swinson DR, Hamilton EB Annals of the rheumatic diseases (1977)
    55. [55]
      Dermatomyositis following rhabdomyolysis.Marks SH, McShane DJ, Mitchell DM The Journal of rheumatology (1976)

    HemoChat

    by SPINAI

    Evidence-based clinical decision support powered by SNOMED-CT, Neo4j GraphRAG, and NASS/AO/NICE guidelines.

    ⚕ For clinical reference only. Not a substitute for professional judgment.

    © 2026 HemoChat. All rights reserved.
    Research·Pricing·Privacy & Terms·Refund·SNOMED-CT · NASS · AO Spine · NICE · GraphRAG