Scleroderma in childhood: a 35-year history of cases and review of the literature.

Shriners Hospital for Children, Honolulu Hospital, Hawaii 96826-1099, USA.

Scleroderma is a connective tissue disease with a variety of clinical presentations. The purpose of our study was to review representative cases of scleroderma in childhood to determine the initial presentation, course of the disease, orthopaedic manifestations, and the necessity and success of surgical intervention. Records from the four medical centers were retrospectively reviewed to identify the patients who were diagnosed with scleroderma over the past 35 years. Data on disease presentation, course, treatment, and response were collected. Seventeen cases representing the variety of clinical presentations are presented. Scleroderma characteristically presents with asymmetric lesions involving the extremities and remains a diagnostic and therapeutic challenge. Treatment must be individualized to the patient and his or her specific limitations brought about by the disease. Early diagnosis, supportive care, and physical therapy combined with early orthopaedic surgical intervention to release joint contractures are among the most efficacious treatments.

PMID: 10912598 [PubMed - indexed for MEDLINE]