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[Scleroderma in children]

A C Frati Munari, G Culebro Nieves, E Velázquez

    Boletin Medico Del Hospital Infantil De Mexico
    |March 1, 1979
    PubMed
    Summary
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    Scleroderma in children presents differently based on type. Localized forms had varied outcomes, while progressive systemic sclerosis (PSS) often involved systemic symptoms and had a poor prognosis despite treatments.

    Area of Science:

    • Pediatric Rheumatology
    • Dermatology
    • Autoimmune Diseases

    Context:

    • Scleroderma encompasses localized and systemic forms, with distinct clinical presentations and prognoses.
    • Pediatric scleroderma requires understanding of disease subtypes and their impact on growth and development.
    • Limited data exists on the long-term outcomes and treatment efficacy for childhood scleroderma.

    Purpose:

    • To review the clinical course and outcomes of pediatric scleroderma patients.
    • To differentiate the progression and systemic involvement between localized scleroderma and progressive systemic sclerosis (PSS) in children.
    • To evaluate the impact of various treatments on disease progression.

    Summary:

    • Fifteen children (10 localized scleroderma, 5 PSS) aged 2-11 years were followed for a mean of 3.4 years.

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  • Localized scleroderma showed varied outcomes, with surgical intervention successful in "en coup de sabre" lesions; systemic symptoms were rare.
  • Progressive systemic sclerosis (PSS) patients exhibited significant extracutaneous manifestations, including pulmonary fibrosis, with one fatality; treatments showed limited efficacy.

    • Impact:

    • Highlights the distinct clinical trajectories of localized versus systemic scleroderma in pediatric populations.
    • Underscores the need for specialized management strategies for pediatric scleroderma subtypes.
    • Suggests current therapeutic interventions may have limited impact on disease course, warranting further research.