TY - JOUR T1 - Efficacy of bosentan in treatment of unresponsive cutaneous ulceration in disabling pansclerotic morphea in children. JF - The Journal of Rheumatology JO - J Rheumatol SP - 2538 LP - 2540 VL - 33 IS - 12 AU - Rosa Roldan AU - Guadalupe Morote AU - Maria del Carmen Castro AU - Maria Dolores Miranda AU - Jose Carlos Moreno AU - Eduardo Collantes Y1 - 2006/12/01 UR - http://www.jrheum.org/content/33/12/2538.abstract N2 - Disabling pansclerotic morphea (PM) of childhood is a rare and debilitating variant of localized scleroderma. We describe a 4-year-old girl with rapid progression of deep cutaneous fibrosis extending into the muscle fascia with disabling joint contractures of the hips, knees, ankles, and fingers and recalcitrant ischemic ulcerations. Within the first months of therapy with dual oral endothelin receptor antagonist bosentan (31.25 mg qd [corrected] for 4 weeks, then 31.25 mg bid) limb ulcers improved, with resolution of the widespread sclerotic skin lesions. Joint mobility improved, and a substantial decrease of skin thickness was noted. No side effects were noted. In the context of other data in scleroderma, bosentan may be a promising option in the treatment of PM. ER -