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Juvenile dermatomyositis: a severe and atypical presentation

Isabel Moitinho de Almeida1, Marta Abreu Andrade1, Miguel Bernardo1

  • 1Hospital de Santa Maria, Lisbon, Portugal.

ARP Rheumatology
|July 9, 2025
PubMed

Insights

This case highlights severe juvenile dermatomyositis (JDM) with atypical skin lesions but significant vasculopathy. Early diagnosis and multidisciplinary treatment are crucial for improving outcomes in JDM patients.

Area of Science:

  • Pediatric Rheumatology
  • Immunology

Background:

  • Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting muscles and skin.
  • This case presents a severe JDM presentation with atypical skin manifestations.

Purpose of the Study:

  • To highlight the importance of timely diagnosis and early treatment initiation in severe JDM.
  • To emphasize the role of a multidisciplinary approach in managing complex JDM cases.

Main Methods:

  • Case report of a 9-year-old girl with severe JDM.
  • Clinical presentation: asthenia, edema, inability to walk, dysphonia, dysphagia, muscle weakness, rash, skin ulcers, anasarca.
  • Diagnostic workup included elevated muscle enzymes, NXP-2 antibody detection, and identification of alveolar hemorrhage and retinal vasculitis.

Main Results:

  • The patient received a combination of prednisolone, methotrexate, IV immunoglobulin, and mycophenolate mofetil.
  • Demonstrated full resolution of myositis, muscle strength recovery, healed skin ulcers, and improved vision.
  • Atypical presentation without typical skin lesions but with severe vasculopathy (retinopathy, alveolar hemorrhage).

Conclusions:

  • JDM can present atypically, emphasizing the need for vigilance beyond classic symptoms.
  • Early diagnosis and prompt, comprehensive treatment are vital for favorable outcomes in severe JDM.
  • A multidisciplinary team approach is essential for managing the multifaceted complications of JDM.
Abstract

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