Scleroderma

Scleroderma is a rare, chronic autoimmune disease characterized by excessive deposition of collagen in the skin and major organs.

• Scleroderma affects approximately 300,000 people in the United States. It is four times more common in women than men.
• There are three main forms of scleroderma: diffuse, limited (CREST syndrome) and linear scleroderma (also known as morphea).
• Diffuse and limited scleroderma are systemic diseases. Linear (localized) scleroderma only affects the skin and can occur at any age.
• It can be treated with topical agents such as corticosteroids, calcineurin inhibitors and imiquimod. If the skin overlying joints is involved, then adjunctive physical therapy is important to preserve joint function.
• Plaquenil (hydroxychloroquine), methotrexate and other immunosuppressive drugs have been used with success in linear scleroderma.
• Ultraviolet light therapy (narrowband UVB, PUVA and UVA-1) are also beneficial for linear scleroderma.
• Diffuse scleroderma can cause musculoskeletal, pulmonary, gastrointestinal, renal and other complications.
• Patients with a large amount of skin involvement is more likely to have involvement of blood vessels and internal organs.
• Impaired lung function is commonly seen in patients with diffuse scleroderma; however, it does not necessarily cause symptoms, such as shortness of breath
• The limited form of systemic scleroderma has a slow onset and progression, with skin hardening confined to the hands and face and milder internal organ involvement.
• Limited scleroderma is referred to as "CREST" syndrome which is an acronym for: Calcinosis, Raynaud's syndrome, Esophageal dysmotility, Sclerodactyly and Telangiectasia.
• Ulceration of the fingertips is a common problem in both forms of systemic scleroderma. Digital ulcerations can be helped by prostacyclin (iloprost) infusion.
• Iloprost being a drug which increases blood flow by relaxing the arterial wall. Immunosuppressant drugs, such as mycophenolate mofetil or cyclophosphamide are sometimes used to slow the progress of scleroderma.