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

Scleroderma is a connective tissue disease with a variety of clinical prese ntations. The purpose of our study was to review representative cases of sc leroderma in childhood to determine the initial presentation, course of the disease, orthopaedic manifestations, and the necessity and success of surg ical intervention. Records from the four medical centers were retrospective ly reviewed to identify the patients who were diagnosed with scleroderma ov er the past 35 years. Data on disease presentation, course, treatment, and response were collected. Seventeen cases representing the variety of clinic al presentations are presented. Scleroderma characteristically presents wit h 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 diagno sis, supportive care, and physical therapy combined with early orthopaedic surgical intervention to release joint contractures are among the most effi cacious treatments.