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The Scleroderma Hand: Manifestations of Disease and Approach to Management
Ariel A Williams1, Hannah M Carl2, Scott D Lifchez1
1Department of Plastic and Reconstructive Surgery, Johns Hopkins Medicine, Baltimore, MD.
Insights
Scleroderma, an autoimmune disease, causes painful hand issues like calcium deposits and digital ischemia. Careful surgical intervention offers the best solution for these challenging, disabling hand conditions.
Area of Science:
- Rheumatology
- Hand Surgery
- Autoimmune Diseases
Background:
- Scleroderma is a rare autoimmune connective tissue disease.
- It frequently impacts the hands, causing significant morbidity.
- Hand manifestations include calcinosis, digital ischemia, and joint contractures.
Purpose of the Study:
- To review the surgical challenges and outcomes for scleroderma patients with hand conditions.
- To highlight the importance of careful surgical planning in this patient population.
Main Methods:
- Review of clinical presentations of scleroderma hand disease.
- Discussion of surgical considerations for patients with compromised vascularity and soft tissue.
Main Results:
- Scleroderma hand conditions present unique surgical challenges due to poor vascularity and tissue deficits.
- Surgical intervention, when meticulously performed, can effectively manage disabling hand manifestations.
- Complications include wound healing issues and potential for infection.
Conclusions:
- Despite challenges, surgery is often the optimal treatment for disabling hand conditions in scleroderma.
- Careful surgical technique and management are crucial for successful outcomes.
Abstract:
Scleroderma is a rare autoimmune connective tissue disorder that often affects the hands. Manifestations in the hands include calcium deposits within the soft tissues that cause pain and may ulcerate through the skin, digital ischemia resulting in chronic wounds and digital gangrene, and joint contracture. Because of the underlying disease, patients with scleroderma have poorly vascularized tissue and a deficient soft tissue envelope, which make surgery particularly challenging. However, when undertaken with care, surgical intervention is often the best option for addressing the disabling hand conditions that so often accompany this disease.
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