title: Localized scleroderma and regional inflammatory myopathy.
abstract: Inflammatory myopathy is rare in localized scleroderma. We report 2 new cases of regional inflammatory myopathy associated with localized scleroderma and review 10 reported cases of localized scleroderma associated with an inflammatory myopathy with regional muscle involvement, more often in the upper extremities. Serum creatine kinase was mildly elevated or normal. Histopathology often showed perimysial inflammation and plasma cell infiltration. These cases demonstrate that inflammatory myopathy should be considered in patients with localized scleroderma and regional muscle weakness, pain or atrophy. Muscle biopsy can confirm the diagnosis of myositis, which if identified, will require anti-inflammatory and/or immunosuppressive therapy.
authors.affiliation: Neurology Service, Department of Veterans Affairs, Pittsburgh, PA 15240, USA; Department of Neurology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA. Electronic address: zivkovics@upmc.edu.
authors.forename: Saša A
authors.initials: SA
authors.lastname: Zivković
authors.affiliation: Department of Neurology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.
authors.forename: William
authors.initials: W
authors.lastname: Freiberg
authors.affiliation: Department of Neurology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA; Department of Pathology (Neuropathology), University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.
authors.forename: David
authors.initials: D
authors.lastname: Lacomis
authors.affiliation: Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.
authors.forename: Robyn T
authors.initials: RT
authors.lastname: Domsic
authors.affiliation: Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Medsger
mesh_terms: D000328:Adult; D002648:Child; D003402:Creatine Kinase / Q000097:blood; D005260:Female; D006801:Humans; D007866:Leg; D008279:Magnetic Resonance Imaging; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology; D009220:Myositis / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D012594:Scleroderma, Localized / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D013904:Thoracic Vertebrae
chemical_list: D003402:Creatine Kinase
keywords: Inflammatory myopathy; Linear scleroderma; Localized scleroderma; Morphea; Perimyositis