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Case Report
Efficacy of Bosentan in Treatment of Unresponsive Cutaneous Ulceration in Disabling Pansclerotic Morphea in Children
ROSA ROLDAN, GUADALUPE MOROTE, MARIA del CARMEN CASTRO, MARIA DOLORES MIRANDA, JOSE CARLOS MORENO, and EDUARDO COLLANTES ABSTRACT. 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 qid 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. (J Rheumatol 2006;33:2538–40) Key Indexing Terms:
BOSENTAN From the Department of Rheumatology and the Department of Dermatology, Reina Sofia Hospital, Cordoba, Spain. R. Roldan, MD; G. Morote, MD; M. del Carmen Castro, MD; M.D. Miranda, MD; E. Collantes, MD, PhD, Department of Rheumatology; J.C. Moreno, MD, Department of Dermatology. Address reprint requests to Dr. R. Roldan, Unidad Clínica de Reumatología, Hospital Universitario Reina Sofia, Av. Menendez Pidal, s/n, ES-14004 Córdoba, Spain. E-Mail: peperosa@teleline.es Accepted for publication July 31, 2006.
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