title: Efficacy of bosentan in treatment of unresponsive cutaneous ulceration in disabling pansclerotic morphea in children.
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 qd for 4 weeks [DOSAGE ERROR CORRECTED], 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.
authors.affiliation: Department of Rheumatology, Reina Sofia Hospital, Cordoba, Spain. peperosa@teleline.es
authors.forename: Rosa
authors.initials: R
authors.lastname: Roldan
authors.forename: Guadalupe
authors.initials: G
authors.lastname: Morote
authors.forename: Maria del Carmen
authors.initials: Mdel C
authors.lastname: Castro
authors.forename: Maria Dolores
authors.initials: MD
authors.lastname: Miranda
authors.forename: Jose Carlos
authors.initials: JC
authors.lastname: Moreno
authors.forename: Eduardo
authors.initials: E
authors.lastname: Collantes
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D002675:Child, Preschool; D003286:Contracture / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D019988:Children with Disabilities; D005260:Female; D006801:Humans; D012594:Scleroderma, Localized / Q000150:complications / Q000188:drug therapy* / Q000469:parasitology; D012883:Skin Ulcer / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D013449:Sulfonamides / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan