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Focal scleroderma and severe cardiomyopathy. Patient report and brief review

E C Moore1, F Cohen, Z Farooki

  • 1Division of Clinical Immunology, Children's Hospital of Michigan, Detroit 48201.

American Journal of Diseases of Children (1960)
|February 1, 1991
PubMed

Insights

This study reports the first case of localized scleroderma with severe heart failure in an infant. Prednisone therapy successfully treated both cardiac and serological abnormalities, highlighting the potential for systemic involvement.

Area of Science:

  • Pediatrics
  • Rheumatology
  • Cardiology

Background:

  • Localized scleroderma is a connective tissue disease typically affecting the skin.
  • Cardiac involvement is rare in localized scleroderma, more commonly seen in systemic sclerosis.
  • Infantile cardiomyopathy presents a significant diagnostic and therapeutic challenge.

Observation:

  • A 21-month-old infant presented with simultaneous localized scleroderma and severe cardiomyopathy.
  • The infant exhibited cardiac dysfunction and elevated inflammatory markers, including rheumatoid factor, IgM, IgG, and erythrocyte sedimentation rate.
  • Skin manifestations of scleroderma were also present.

Findings:

  • Prednisone therapy led to the normalization of cardiac function and serological abnormalities.
  • Significant, though incomplete, resolution of skin lesions was observed concurrently.
  • This case represents the first documented instance of clinically significant cardiac involvement in localized scleroderma.

Implications:

  • Localized scleroderma may be associated with serious internal organ involvement, particularly cardiac disease.
  • Early recognition and treatment of cardiac complications are crucial in pediatric scleroderma cases.
  • This case underscores the importance of considering systemic manifestations in patients with focal scleroderma.

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