BACKGROUND Scleroderma is an autoimmune disease with a characteristic vascular pathology. The vasculopathy associated with scleroderma is one of the major contributors to the clinical manifestations of the disease. METHODOLOGY/PRINCIPAL FINDINGS We used immunohistochemical and mRNA in situ hybridization techniques to characterize this vasculopathy and showed with morphometry that scleroderma ...

The principal effect of TGF-beta1 on mesenchymal cells is its stimulation of ECM synthesis. Previous reports indicated the significance of the autocrine TGF-beta loop in the pathogenesis of scleroderma. In this study, we focused on Smad7 and Smurfs, principal molecules in the negative regulation of TGF-beta signaling, to further understand the autocrine TGF-beta loop in scleroderma. Scleroderma...

BACKGROUND Scleroderma skin overexpresses the platelet-derived growth factor receptor beta-subunit (PDGFR-beta) in dermal vessels and PDGFR-beta messenger RNA in cultured fibroblasts. Moreover, increased levels of PDGF and stimulatory autoantibodies to PDGFR have been identified in the serum of scleroderma patients. OBJECTIVE Imatinib being an inhibitor of tyrosine kinase receptors such as PD...

This study examined associations between childbearing and risk of scleroderma by using national population-based registry data from Sweden. Women with a discharge diagnosis of scleroderma from 1964 to 1999 (n = 2,149) were identified in the Swedish Inpatient Register. These cases were matched by year and month of birth and region of residence to as many as five controls obtained from the Multi-...

Skin blood flow was measured by the clearance of radioactive xenon ((133)Xe) injected intracutaneously in eight patients with scleroderma and nine control subjects under conditions of controlled temperature and humidity. Scleroderma patients, on being cooled 1 hr at 18 degrees C, had a rate constant of (133)Xe clearance from the dorsal finger skin which was 0.04 +/-0.07 min(-1) (mean +/-SD), co...

Exposure to solvents has been reported to increase the risk of scleroderma. The authors investigated the relation between exposures to solvents in occupational and hobby settings and the development of scleroderma among women in a case-control study with population-based controls in Michigan (1980-1991) and Ohio (1980-1992). A total of 660 cases and 2,227 frequency-matched controls were intervi...

Scleroderma is an autoimmune disease characterized by extracellular matrix deposition and inflammation. Topical vitamin D analogs have been reported as effective treatments for scleroderma. We previously reported that a matricellular protein, periostin (POSTN), contributes to pathogenesis of scleroderma as POSTN knockout mice were resistant to bleomycin (BLM)-induced scleroderma. We investigate...

how to cite this article: allmendinger am, ricci ja, desai ns, viswanadhan n, rodriguez d. atypical neuroimaging manifestations of linear scleroderma “en coup de sabre”. iran j child neurol. summer 2015;9(3):62-68. abstract linear scleroderma “en coup de sabre” is a subset of localized scleroderma with band-like sclerotic lesions typically involving the fronto-parietal regions of the scalp. pat...

Scleroderma (systemic sclerosis) is a connective tissue disorder which involves skin, gastrointestinal tract, kidneys and lungs with considerable morbidity. Scleroderma renal crisis is a well-known severe form of renal involvement, characterised by the presence of high blood pressure with variable degrees of renal insufficiency. We report a case of scleroderma renal crisis diagnosed on the basi...