Summary
Morphea is an uncommon but potentially disabling condition. Abnormalities in immune system parameters, endothelial activation and fibroblast metabolism have been described, but a unifying pathophysiologic model remains to be tested. The clinician is faced with considerable uncertainty when choosing a treatment modality for LS. Given the benign natural progression of plaque type morphea, treatment with topical modalities such as superpotent corticosteroids or calcipotriol is prudent. For more generalized forms of morphea, as well as the linear forms, UVA is currently the best-documented therapeutic modality. In the absence of access to UVA treatment, oral calciferol or systemic immunosuppression may be contemplated. Recent studies support the use of methotrexate with systemic corticosteroids for the management of aggressive disease. The authors have had variable success with PUVA, broadband UVA, and methotrexate in severe disease. Resolution of therapeutic uncertainty must await the organization and completion of controlled trials.
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Kellet, C.V., Orteu, C.H., Dutz, J.P. (2011). Localized Scleroderma. In: Hertl, M. (eds) Autoimmune Diseases of the Skin. Springer, Vienna. https://doi.org/10.1007/978-3-211-99225-8_7
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