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Scleroderma affects
approximately 300,000 people in the United
States. It is four times more common in women
than men. |
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There are three main
forms of scleroderma: diffuse, limited (CREST
syndrome) and linear scleroderma (also known
as morphea). |
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Diffuse and limited
scleroderma are systemic diseases. Linear
(localized) scleroderma only affects the skin
and can occur at any age. |
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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.
|
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Plaquenil (hydroxychloroquine),
methotrexate and other immunosuppressive drugs
have been used with success in linear scleroderma.
|
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Ultraviolet light therapy
(narrowband UVB, PUVA and UVA-1) are also
beneficial for linear scleroderma. |
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Diffuse scleroderma
can cause musculoskeletal, pulmonary, gastrointestinal,
renal and other complications. |
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Patients with a large
amount of skin involvement is more likely
to have involvement of blood vessels and internal
organs. |
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Impaired lung function
is commonly seen in patients with diffuse
scleroderma; however, it does not necessarily
cause symptoms, such as shortness of breath |
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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. |
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Limited scleroderma
is referred to as "CREST" syndrome
which is an acronym for: Calcinosis, Raynaud's
syndrome, Esophageal dysmotility, Sclerodactyly
and Telangiectasia. |
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Ulceration of the fingertips
is a common problem in both forms of systemic
scleroderma. Digital ulcerations can be helped
by prostacyclin (iloprost) infusion. |
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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. |
