Juvenile dermatomyositis: common manifestations of a rare disease

  • Mariana Capela Department of Pediatrics, Centro Hospitalar Vila Nova de Gaia/Espinho
  • Joana Reis Department of Pediatrics, Centro Hospitalar Vila Nova de Gaia/Espinho
  • Diana Soares Department of Pediatrics, Centro Hospitalar Vila Nova de Gaia/Espinho
  • Andreia Ribeiro Department of Pediatrics, Centro Hospitalar Vila Nova de Gaia/Espinho
  • Isabel Pinto Pais Department of Pediatric Gastroenterology, Centro Hospitalar Vila Nova de Gaia/Espinho
  • Lúcia Trindade Rodrigues Department of Pediatric Reumatology, Centro Hospitalar Vila Nova de Gaia/Espinho
Keywords: exanthema, juvenile dermatomyositis, myositis

Abstract

Juvenile dermatomyositis is an autoimmune systemic vasculopathy, mainly characterized by chronic inflammation of the striated muscle and skin. The authors report the case of a 15-year-old boy presenting with a four-month history of myalgia and proximal muscle weakness on the upper and lower limbs. These symptoms were associated with heliotrope palpebral exanthema, erythema in the dorsum of the hands, Gottron’s papules, erythematous and petechial rash on the extensor face of the thighs, and mild dysphagia for solids. Blood tests revealed an increase in muscle enzymes and electromyography showed changes suggestive of severe acute myopathy. Intravenous methylprednisolone was initiated, followed by a combination regimen of prednisolone and methotrexate. Progressive dysphagia, cutaneous abnormality, and muscular strength improvement were noted. With this case, the authors intend to raise awareness of a rare disease with an essentially clinical diagnosis, presenting in most cases with characteristic manifestations that should be recognised.

Author Biographies

Mariana Capela, Department of Pediatrics, Centro Hospitalar Vila Nova de Gaia/Espinho

MD, Pediatrics Department

Joana Reis, Department of Pediatrics, Centro Hospitalar Vila Nova de Gaia/Espinho

MD, Pediatrics Department

Diana Soares, Department of Pediatrics, Centro Hospitalar Vila Nova de Gaia/Espinho

MD, Pediatrics Department

Andreia Ribeiro, Department of Pediatrics, Centro Hospitalar Vila Nova de Gaia/Espinho

MD, Pediatrics Department

Isabel Pinto Pais, Department of Pediatric Gastroenterology, Centro Hospitalar Vila Nova de Gaia/Espinho

MD, Pediatric Gastroenterology Department

Lúcia Trindade Rodrigues, Department of Pediatric Reumatology, Centro Hospitalar Vila Nova de Gaia/Espinho

MD, Pediatric Rheumatology Department

References

Rider GL, Katz JD, Jones OU. Developments in the Classification and Treatment of the Juvenile Idiopathic Inflammatory Myopathies. Rheum Dis Clin North Am. 2013; 39:877-904.

Prevalence and incidence of rare diseases: Bibliographic data. Orphanet Report Series. Rare Diseases Collection. January 2018.

Rider GL, Lindsley CB, Miller FW. Juvenile Dermatomyosites. Textbook of Pediatric Rheumatology. Chapter 26, p. 351-83. Elsevier, Seventh edition.

Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975; 292:344-7.

Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975; 292:403-7.

Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser M, et al. Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups. European League Against Rheumatism/American College of Rheumatology Arthritis Rheumatol. 2017; 69:2271­82.

Enders FB, Bader- Meunier B, Baildam E, Constantin T, Dolezalova P, Feldman MF, et al. Consensus-based recommendations for the management of juvenile dermatomyositis. Ann Rheum Dis 2017; 76:329–40.

Ruperto N, Pistorio A, Oliveira S, Zulian F, Cuttica R, Ravelli A, et al. Paediatric Rheumatology International Trials

Organisation (PRINTO). Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial. Lancet 2016; 13;387:671-8.

Inocencio J, Enríquez-Merayo E, Casado R, González-Granado LI. Subcutaneous Immunoglobulin in Refractory Juvenile Dermatomyositis. Pediatrics. 2016; 137. pii: e20153537.

Fasano S, Gordon P, Hajji R, Loyo E, Isenberg DA. Rituximab in the treatment of inflammatory myopathies: a review. Rheumatology (Oxford). 2016; 2.pii: kew146.

Moghadam-Kia, Oddis CV, Aggarwal R. Modern Therapies for Idiopathic Inflammatory Myopathies (IIMs): Role of Biologics. Clin Rev Allergy Immunol. 2017; 52:81-7.

Chojnowski MM, Felis-Giemza A, Olesińska M. Capillaroscopy – a role in modern rheumatology. Reumatologia 2016; 54:67–72.

Gunawardena H, Betteridge ZE, McHugh NJ. Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression. Rheumatology (Oxford). 2009; 48:607-12.

Holden DJ, Brownell AKW, Fritzler MJ. Clinical and serological features of patients with polymyositis or dermatomyositis. Can Med Assoc J. 1985; 132:649–53.

Carbonell MS, Antoli HC, Fonseca AL, Talayero JM, Tomás PE. Dermatomyositis. Presentation of a mild to moderate case with early dysphagia. An Pediatr (Barc) 2015; 82:e86-9.

Published
2020-08-07
How to Cite
Capela, M., Reis, J., Soares, D., Ribeiro, A., Pais, I. P., & Rodrigues, L. T. (2020). Juvenile dermatomyositis: common manifestations of a rare disease. NASCER E CRESCER - BIRTH AND GROWTH MEDICAL JOURNAL, 29(3), 149-152. https://doi.org/10.25753/BirthGrowthMJ.v29.i3.14261
Section
Case Reports