Treatment varies depending on the patient’s disease activity, lesion location and extent, and whether there are related problems. Careful clinical evaluation is the primary method for monitoring scleroderma. X-rays and computerized tomography (CT) scans are used to look at bone abnormalities. Thermography can detect differences in skin temperature between the lesion and normal tissue. Ultrasound and magnetic resonance imaging (MRI) can aid soft tissue assessment.
Current treatment is focused on controlling inflammation, as this decreases the risk of serious problems such as differences in limb length, areas of sunken skin on the face, limited joint movement, and damage to internal organs. Patients with linear scleroderma that involve lesions on the head, deep lesions, or widespread disease, are usually treated with systemic medications that suppress the immune system. These medicines include methotrexate which is given by injection or taken by mouth once a week, and corticosteroids, which are taken by mouth (prednisone) or given by infusion, or medication administered through a vein (I.e., intravenous methylprednisolone).
One randomized clinical trial showed that methotrexate was better than placebo at maintaining disease control after initial corticosteroid treatment. Other immunosuppressive medications include mycophenolate mofetil, cyclosporine, and tacrolimus. More work is needed to determine the best therapy for localized scleroderma. Immunosuppressive medicines can increase a patient’s risk for developing an infection and have other possible side effects. These will be reviewed by your doctor.
For patients with mild superficial disease, topical medications are often used to control inflammation and softening the skin. These medications include topical corticosteroids, calcipotriene, tacrolimus, pimecrolimus, and imiquimod. Moisturizers may help protect and soften the skin.
Phototherapy has been used to treat patients with widespread, superficial skin disease. Both UVB and UVA have been reported to help. More study is needed to evaluate the potential side effects from exposing children to large amounts of ultraviolet light.
Physical and occupational therapy to improve strength and function is important for patients with muscle weakness, limb length differences, and limited joint movement. Early referral to therapy should be considered in order to prevent loss of joint motion and function.
Surgery is not recommended during treatment of active disease. Surgery may be needed for patients with severe pain or limitation, and can improve the appearance of patients with severe facial lesions. However, because the skin is abnormal there can be poor wound healing, and surgery may trigger a flare of disease in some cases. Cosmetic makeup can be used to make the lesions less noticeable.
There is no known cure for localized scleroderma. The disease can stop on its own (remission), but the timing of this varies. Circumscribed morphea lesions that do not extend into deeper tissues may go into remission within a few years, while linear scleroderma lesions - especially on the head or scalp - can remain active for many years or even throughout childhood. It is important that patients who have lesions involving deeper tissues continue to be monitored on a quarterly basis. Once treatment is stopped, patients should be monitored at least yearly because the disease may come back (relapse or flare).