Francesco Zulian1. 1. Division of Paediatric Rheumatology, Department of Paediatrics, University of Padova, Padova, Italy.
Abstract
PURPOSE OF REVIEW: Localized scleroderma, also known as morphea, is the most frequent form of scleroderma in childhood. Early diagnosis, appropriate assessment and effective treatment may improve the long-term outcome. RECENT FINDINGS: Recent studies, regarding the microchimerism theory or the mechanism of action of phototherapy, have yielded important information on the disease etiopathogenesis. Others have added interesting contributions on new outcome measures for the disease assessment and for the development of future therapeutic trials. Previous results, using methotrexate and phototherapy, have been confirmed. A successful use of bosentan, an endothelin receptor antagonist with vasodilatative and antifibrotic properties for refractory cutaneous ulcerations in pansclerotic morphea, opens new horizons of treatment. SUMMARY: Studies over the past year highlight the role of some outcome measures in the disease assessment and monitoring, with important implications both for the clinical practice and future clinical trials.
PURPOSE OF REVIEW: Localized scleroderma, also known as morphea, is the most frequent form of scleroderma in childhood. Early diagnosis, appropriate assessment and effective treatment may improve the long-term outcome. RECENT FINDINGS: Recent studies, regarding the microchimerism theory or the mechanism of action of phototherapy, have yielded important information on the disease etiopathogenesis. Others have added interesting contributions on new outcome measures for the disease assessment and for the development of future therapeutic trials. Previous results, using methotrexate and phototherapy, have been confirmed. A successful use of bosentan, an endothelin receptor antagonist with vasodilatative and antifibrotic properties for refractory cutaneous ulcerations in pansclerotic morphea, opens new horizons of treatment. SUMMARY: Studies over the past year highlight the role of some outcome measures in the disease assessment and monitoring, with important implications both for the clinical practice and future clinical trials.
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