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Juvenile dermatomyositis.

V Seth1, S K Kabra, O P Semwal

  • 1Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi.

Indian Journal of Pediatrics
|May 1, 1996
PubMed
Summary
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This study details 7 pediatric cases, noting a female predominance and a median onset age of 6 years. All patients showed skin rash, muscle weakness, and abnormal enzyme levels, responding well to corticosteroid treatment.

Area of Science:

  • Pediatric Rheumatology
  • Neuromuscular Disorders
  • Dermatomyositis

Background:

  • Idiopathic inflammatory myopathies (IIMs) are rare in children.
  • Dermatomyositis is the most common IIM in the pediatric population.
  • Understanding the clinical presentation and treatment response is crucial for early diagnosis and management.

Purpose of the Study:

  • To describe the clinical profile and follow-up of pediatric patients with a specific neuromuscular disorder.
  • To analyze the diagnostic findings, including enzyme profiles, muscle biopsies, and electromyograms (EMG).
  • To evaluate the efficacy of corticosteroid therapy in this cohort.

Main Methods:

  • Retrospective case series of 7 children.
  • Clinical data collection including age of onset, symptoms (skin rash, muscle weakness), and laboratory findings (enzyme profile).

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  • Diagnostic investigations included muscle biopsy and electromyogram (EMG).

    • Main Results:

    • Female predominance observed (female to male ratio 1:6).
    • Median age of onset was 6 years.
    • All patients presented with skin rash, muscle weakness, and abnormal enzyme profiles. Muscle biopsy and EMG were abnormal in most patients.
    • All children responded favorably to corticosteroid treatment, with two showing significant improvement after intravenous dexamethasone bolus therapy.

    Conclusions:

    • Pediatric inflammatory myopathies present with characteristic skin and muscle involvement.
    • Early diagnosis supported by enzyme profiles, muscle biopsy, and EMG is essential.
    • Corticosteroids, including intravenous dexamethasone, are effective treatments for pediatric cases.