Question

Please discuss the treatment options for linear scleroderma. My understanding is that there is no clear-cut consensus on optimal therapy and that it can be difficult to assess long-term efficacy because there may be spontaneous regression after several years. Any thoughts on use of calcitriol (Rocaltrol) or MSM (the oral analog of dimethylsulfoxide [DMSO])?

Response from Robert Fox, MD

Localized scleroderma can be divided into 3 main subtypes: morphea, linear scleroderma, and generalized morphea. Plaque morphea usually has a good prognosis and often does not require treatment. Linear scleroderma, whether involving an extremity or the face, is often associated with serologic abnormalities.[1] Although progression of linear to systemic scleroderma is uncommon, the lesions may lead to cosmetic problems due to thickening and induration of the skin.[2]

Linear scleroderma is a connective tissue disorder that characteristically causes skin induration and pigmentary changes in a linear distribution. No therapy is universally accepted for the fibrotic stage of scleroderma. Recently, a beneficial effect of oral calcitriol (1,25-dihydroxyvitamin D3) treatment was reported to be effective in the early stages.[3] During the treatment, dietary calcium intake should be restricted. Calcium, inorganic phosphate, creatinine, and urea in the serum and urine need to be monitored. Five of the seven pediatric patients in one series showed good to excellent improvement of their lesions.[3] One of them had a partial relapse after 19 months but showed an excellent response to a second therapy session with calcitriol. One patient with rapidly progressive disease failed to respond to therapy.

Other suggested treatments include highly potent corticosteroids and psoralen UVA therapy.[4,] 5] An anti-mast cell drug used in the treatment of patients with allergies (N-[3',4'-dimethoxycinnamoyl] anthranilic acid [tranilast, Rizaben]) was reported effective in localized scleroderma in several cases.[6]

The use of DMSO and its analogues have not been reported as beneficial in controlled studies.[7,8,9]

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