Wednesday, December 3, 2008 - 7:21PM EST

Treatment Options for Scleroderma

Localized Scleroderma

Disease activity usually stops spontaneously over time. Treatment is important to reduce disabilities caused by active phases of disease. Treatments used for the treatment of localized scleroderma include:

  • PUVA therapy
  • Topical photodynamic therapy
  • Drug therapy

PUVA Therapy

PUVA therapy or psoralen photochemotherapy refers to treatment with drugs known as "psoralens" plus ultraviolet A (UVA) light. During this treatment the patient is first exposed to psoralens (drugs containing chemicals that react with ultraviolet light) and then to UVA light from a special lamp. PUVA therapy is used to treat several skin conditions including vitiligo and psoriasis. PUVA therapy is used for the treatment of localized scleroderma because studies have shown a significant reduction of collagen production with this treatment. PUVA therapy can be performed in a doctor's office on an outpatient basis. The initial step in this treatment involves administering psoralen to the patient. Psoaralen may be administered either orally or topically (applying it to the skin). This is followed by a timed exposure to ultraviolet light from a special lamp. In one study, PUVA therapy relieved symptoms in about 75% of patients with localized scleroderma.

Topical Photodynamic Therapy

Topical photodynamic therapy (PDT) refers to the use of photochemical reactions involving a photosensitizer agent, light, and oxygen for the treatment of various malignant or benign skin conditions. Topical photodynamic therapy may also be used for the treatment of localized scleroderma. This treatment modality is performed in two steps:

  • Step 1 - A photosensitizing agent such as 5- aminolevulinic acid is applied to the skin of the patient for about 3-6 hours during which time it is taken up by the target cells.

  • Step 2 - This involves activation of the photosensitizer in the presence of oxygen with a specific wavelength of light focused on the target tissue (skin). When the photosensitizer has been activated by exposure to light, a chemical reaction takes place resulting in damage to the target cells. In the case of localized scleroderma, the target is collagen producing cells. The photosensitizer is preferentially taken up by actively dividing collagen producing cells and activation of the photosensitizer results in damage to the collagen producing cells with reduced production of collagen.

Topical photodynamic therapy is performed once or twice weekly for 3 to 6 months. The treatment is usually well-tolerated although it is associated with some side-effects which may include:

  • During exposure to the light source, patients may experience burning, stinging, itching, or pain.

  • Localized swelling and redness of the skin at the area of light exposure.

  • Areas of skin may become darker (hyperpigmentation) or lighter (hypopigmentation). This effect is usually temporary.

Drug Therapy

As mentioned previously, there is no cure for localized scleroderma and drug therapy is usually reserved for patients with active, widespread, advanced disease. Drug therapy may be used to stop the progression of localized scleroderma if the disease involves a large area of the body such as an entire arm or leg. To date, no single drug has been proven to be effective for the treatment of localized scleroderma in controlled clinical trials. Nevertheless, many doctors may still use drug therapy in an attempt to control the symptoms of the disease and/or to prevent further progression of the disease. Some of the drugs that may be used include:

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