Juvenile dermatomyositis – role of the family physician in the approach of a rare disease

Authors

  • Marisa Loio Interna de Formação Específica na USF Santa Clara http://orcid.org/0000-0002-0673-1897
  • André Salgueiro Interno de Formação Específica na USF Santa Clara
  • Helena Cruz Interna de Formação Específica na USF Santa Clara

DOI:

https://doi.org/10.5712/rbmfc12(39)1418

Keywords:

Dermatomyositis. Primary Health Care.Rare Diseases

Abstract

Juvenile dermatomyositis (JDM) is a rare, multisystem, autoimmune disease, characterized by skin and striated muscle vasculitis. In children is the most frequent idiopathic inflammatory myopathy. This case consists in an 11-year old female patient consulted her Family and Community Physician (FCP) with maculopapular rash of the dorsal surface of the metacarpophalangeal and interphalangeal joints of the hands, malar erythema, macular lesions on the extensor surface of the elbows and knees, and reduced muscle strength of the limbs. The fact that the FCP have been aware of these clinical manifestations and made an early referral to Rheumatology for suspected dermatomyositis, allowed a prompt treatment, and a decrease impact of the disease. After making a diagnosis, FCP continues with an active role in the identification of the disease complications and assessing their impact on family dynamics.

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References

Compeyrot-Lacassagne S, Feldman BM. Inflammatory myopathies in children. Pediatr Clin North Am. 2005;52(2):493-520. DOI: http://dx.doi.org/10.1016/j.pcl.2005.01.004 DOI: https://doi.org/10.1016/j.pcl.2005.01.004

Escorial Briso-Montiano M, Solís Sánchez P, Baeza Velasco M, Alonso Rubio A, de Gregorio Alvarez Z. Dermatomiositis amiopática juvenil y calcinosis. An Pediatr (Barc). 2005;62(3):286-8. DOI: http://dx.doi.org/10.1157/13071846 DOI: https://doi.org/10.1157/13071846

Pachman ML. Juvenile Dermatomyositis. In: Behrman RE, Kliegman RM, Jenson HB. Nelson Textbook of Pediatrics. 17th ed. Philadelphia: WB Saundersy; 2005. p. 813-6.

Pilkington C. Juvenile Dermatomyositis. In: Cimaz R, Lehman T, eds. Handbook of Systemic Autoimmune Diseases. Oxford: Elsevier; 2008. p. 89-98. DOI: https://doi.org/10.1016/S1571-5078(07)06011-4

Rider LG, Lindsley CB, Miller FW. Juvenile dermatomyositis. In: Petty RE, Laxer RM, Lindsley CB, Wedderburn LR. Textbook of Pediatric Rheumatology. 7th ed. Philadelphia: Elsevier Saunders; 2016. p. 351-83. DOI: https://doi.org/10.1016/B978-0-323-24145-8.00026-0

Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292(7):344-7. DOI: http://dx.doi.org/10.1056/NEJM197502132920706 DOI: https://doi.org/10.1056/NEJM197502132920706

Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med. 1975;292(8):403-7. DOI: http://dx.doi.org/10.1056/NEJM197502202920807 DOI: https://doi.org/10.1056/NEJM197502202920807

Wedderburn LR, Li CK. Paediatric idiopathic inflammatory muscle disease. Best Pract Res Clin Rheumatol. 2004;18(3):345-58. DOI: http://dx.doi.org/10.1016/j.berh.2004.01.003 DOI: https://doi.org/10.1016/j.berh.2004.01.003

Feldman BM, Rider LG, Reed AM, Pachman LM. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet. 2008;371(9631):2201-12. DOI: http://dx.doi.org/10.1016/S0140-6736(08)60955-1 DOI: https://doi.org/10.1016/S0140-6736(08)60955-1

Iorizzo LJ 3rd, Jorizzo JL. The treatment and prognosis of dermatomyositis: an updated review. J Am Acad Dermatol. 2008;59(1):99-112. DOI: http://dx.doi.org/10.1016/j.jaad.2008.02.043 DOI: https://doi.org/10.1016/j.jaad.2008.02.043

Buckley L, Guyatt G, Fink HA, Cannon M, Grossman J, Hansen KE, et al. 2017 American College of Rheumatology Guideline for the Prevention and Treatment of Glucocorticoid-Induced Osteoporosis. Arthritis Rheumatol. 2017;69(8):1521-37. DOI: http://dx.doi.org/10.1002/art.40137 DOI: https://doi.org/10.1002/art.40137

Ruperto N, Ravelli A, Murray KJ, Lovell DJ, Andersson-Gare B, Feldman BM, et al.; Paediatric Rheumatology International Trials Organization (PRINTO); Pediatric Rheumatology Collaborative Study Group (PRCSG). Preliminary core sets of measures for disease activity and damage assessment in juvenile systemic lupus erythematosus and juvenile dermatomyositis. Rheumatology (Oxford). 2003;42(12):1452-9. DOI: http://dx.doi.org/10.1093/rheumatology/keg403 DOI: https://doi.org/10.1093/rheumatology/keg403

Niewold TB, Wu SC, Smith M, Morgan GA, Pachman LM. Familial aggregation of autoimmune disease in juvenile dermatomyositis. Pediatrics. 2011;127(5):e1239-46. DOI: http://dx.doi.org/10.1542/peds.2010-3022 DOI: https://doi.org/10.1542/peds.2010-3022

Published

2018-02-08

How to Cite

1.
Loio M, Salgueiro A, Cruz H. Juvenile dermatomyositis – role of the family physician in the approach of a rare disease. Rev Bras Med Fam Comunidade [Internet]. 2018 Feb. 8 [cited 2024 Jul. 22];12(39):1-8. Available from: https://rbmfc.org.br/rbmfc/article/view/1418

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Section

Clinical Cases

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