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Scleroderma

Description

An in-depth report on the causes, diagnosis, treatment, and prevention of scleroderma.

Alternative Names

Systemic sclerosis

Treatment for Skin Thickening

Nitroglycerin is a rapidly acting nitrate and is used as an ointment (Nitro-Bid, Nitrol, Nitrong, Nitrostat) to treat hardened skin. Before applying it, any ointment that remains from the previous application should be removed.

Phototherapy

UVA-1 Phototherapy. Phototherapy (light therapy) is now considered by some experts to be the treatment of choice for local scleroderma. Specifically, doctors favor an approach called ultraviolet A-1 (UVA-1) radiation. This treatment produces long UVA wave lengths that do not cause sunburn and may actually repair DNA in damaged skin cells. Research suggests that UVA-1 therapy blocks inflammatory immune factors and the process leading to over-production of collagen, addressing the underlying mechanisms of scleroderma. The procedure is effective for all stages of morphea. It increases skin elasticity and in some cases, achieves complete clearance of symptoms. In one small study, patients with localized scleroderma received 30 treatments over a period of 12 weeks. In a majority of the patients, 80% of the skin patches disappeared or significantly improved. There were no side effects.

UVA-1 phototherapy is quite expensive and requires a special light source not readily available everywhere. In addition, studies are reporting an increased risk with UVA radiation. Whether this applies to UVA-1 phototherapy is not yet clear. Nonetheless, phototherapy is still an effective and important treatment of scleroderma. It may prove to be even more beneficial when combined with certain medications, such as calcipotriene (Dovonex), a form of vitamin D3.

PUVA. An alternative phototherapy regimen called PUVA uses drugs taken by mouth known as psoralens before UVA treatment. It has been used for other skin diseases, including psoriasis. It may prove to be useful for patients with early-onset diffuse scleroderma. In one study, most of those treated with PUVA for 2 days a month for up to 8 years experienced improvement or stabilization in nearly all scleroderma symptoms. Tests for kidney function remained normal. This treatment is known to increase the risk for skin cancer.

Phototherapy with Psoralen Water Bath. Yet another procedure uses UVA light therapy after patients take a bath containing a solution of the psoralen 8-methoxypsoralen (8-MOP). It is safe and well tolerated, although benefits appear to be minor and occur only in a small subset of patients.

Extracorporeal Photopheresis. Another phototherapy treatment under investigation is called extracorporeal photopheresis. It involves withdrawing the patient's blood and treating it with ultraviolet light. Little data exists on its effectiveness, and experts do not recommend it at this time. Still, some experts argue that some initial promise in its use warrants more research.

Vitamin D3 Analogs

A form of vitamin D3, calcipotriene (Dovonex), appears to help block skin cell production. This vitamin is also called calcipotriol in Europe. It also has anti-inflammatory properties and is being investigated as a rub-on treatment and a form taken by mouth for local scleroderma. It may prove to be beneficial when combined with low-dose ultraviolet A1 phototherapy.

Immunosuppressive Agents

D-penicillamine is proving to be an effective agent for softening skin and reducing thickness. (Improvements in thickness with this drug have also been associated with improved survival.)

Methotrexate (Rheumatrex) is another agent commonly used may be even more effective than penicillamine.

Corticosteroids taken by mouth, such as prednisolone and prednisone, are also often employed.

• Review Date: 12/14/2006
• Reviewed By: Harvey Simon, MD, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital

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