title: Localized scleroderma progressing to systemic disease. Case report and review of the literature.
abstract: We describe a 15-year-old girl with biopsy-proven morphea who developed progression to systemic disease 2 years after initial presentation. In contrast to other reported patients with localized scleroderma, some of whom have had mild, nonprogressive systemic involvement, this patient developed severe, debilitating disease, with skin tightness, sclerodactyly, esophageal involvement, restrictive pulmonary disease, and myopathy. From the time of her initial evaluation, the patient was positive for antinuclear antibodies (ANA), which were shown to be primarily directed against the Ku antigens. This observation suggests that ANA may be a prognostic indicator for progression to systemic disease.
authors.affiliation: Division of Rheumatology, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: N
authors.initials: N
authors.lastname: Birdi
authors.forename: R M
authors.initials: RM
authors.lastname: Laxer
authors.forename: P
authors.initials: P
authors.lastname: Thorner
authors.forename: M J
authors.initials: MJ
authors.lastname: Fritzler
authors.forename: E D
authors.initials: ED
authors.lastname: Silverman
mesh_terms: D000974:Antibodies, Antinuclear / Q000276:immunology; D034961:Antigens, Nuclear; D001324:Autoantigens / Q000276:immunology; D015415:Biomarkers; D001706:Biopsy; D002648:Child; D004265:DNA Helicases; D004268:DNA-Binding Proteins / Q000276:immunology; D005260:Female; D006801:Humans; D015151:Immunoblotting; D000072200:Ku Autoantigen; D009687:Nuclear Proteins / Q000276:immunology; D012594:Scleroderma, Localized / Q000175:diagnosis / Q000276:immunology*; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000276:immunology*; D012867:Skin / Q000473:pathology
chemical_list: D000974:Antibodies, Antinuclear; D034961:Antigens, Nuclear; D001324:Autoantigens; D015415:Biomarkers; D004268:DNA-Binding Proteins; D009687:Nuclear Proteins; D004265:DNA Helicases; C106893:XRCC5 protein, human; C000606509:Xrcc6 protein, human; D000072200:Ku Autoantigen