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scleroderma and skin and treatment

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https://www.readbyqxmd.com/read/28933138/assessment-of-treatment-effects-on-digital-ulcer-and-blood-perfusion-by-laser-speckle-contrast-analysis-in-a-patient-affected-by-systemic-sclerosis
#1
B Ruaro, S Paolino, C Pizzorni, M Cutolo, A Sulli
Laser speckle contrast analysis (LASCA) is a good tool to evaluate the variation in peripheral blood perfusion during long-term follow-up and is able to safely monitor digital ulcer evolution in scleroderma patients. It evaluates blood perfusion in different areas within the skin lesions and surrounding them during standard treatment.
September 21, 2017: Reumatismo
https://www.readbyqxmd.com/read/28899803/humanistic-and-cost-burden-of-systemic-sclerosis-a-review-of-the-literature
#2
REVIEW
Aryeh Fischer, Evelina Zimovetz, Caroline Ling, Dirk Esser, Nils Schoof
BACKGROUND: Systemic sclerosis (SSc), or systemic scleroderma, is a chronic multisystem autoimmune disease characterised by widespread vascular injury and progressive fibrosis of the skin and internal organs. Patients with SSc have decreased survival, with pulmonary involvement as the main cause of death. Current treatments for SSc manage a range of symptoms but not the cause of the disease. Our review describes the humanistic and cost burden of SSc. METHODS: A structured review of the literature was conducted, using predefined search strategies to search PubMed, Embase, and the Cochrane Library...
September 9, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28885215/hyperspectral-imaging-as-a-novel-diagnostic-tool-in-microcirculation-of-wounds
#3
Georg Daeschlein, Inga Langner, Thomas Wild, Sebastian von Podewils, Claudia Sicher, Thomas Kiefer, Michael Jünger
Monitoring of perfusion is a cornerstone in surgery, phlebology and basic science to proof wound healing by interventions. In chronic wound management it is of utmost importance to realize and parametrize wound bed perfusion to verify actual, and plan further treatment by noninvasive diagnostics. Up to now monitoring is based on visual inspection of wounds as conventionally practiced over more than decades. The main problems of visual inspection are the lack of standardization and comparability because of interindividual variations...
September 4, 2017: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/28872977/growth-differentiation-factor-15-gdf-15-level-and-relation-to-clinical-manifestations-in-egyptian-systemic-sclerosis-patients-preliminary-data
#4
Sherif M Gamal, Fatema T Elgengehy, Asmaa Kamal, Samah A El Bakry, Elham Shabaan, Aliaa Elgendy, Iman H Bassyouni
AIM OF THE WORK: This study aims to assess Growth differentiation factor-15 (GDF-15) level in Scleroderma patients and its relation to disease manifestations. PATIENTS AND METHODS: This study included 55 scleroderma patients and 40 age and sex matched healthy volunteers. All patients were subjected to full history taking, thorough clinical examination, and laboratory investigations. GDF-15 serum levels were analyzed in patients and controls using human GDF-15 immunoassay Quantikine ELISA kit...
October 2017: Immunological Investigations
https://www.readbyqxmd.com/read/28870693/therapeutic-targeting-of-taz-and-yap-by-dimethyl-fumarate-in-systemic-sclerosis-fibrosis
#5
Tetsuo Toyama, Agnieszka P Looney, Brendon M Baker, Lukasz Stawski, Paul Haines, Robert Simms, Aleksander D Szymaniak, Xaralabos Varelas, Maria Trojanowska
Systemic sclerosis (scleroderma, SSc) is a devastating fibrotic disease with few treatment options. Fumaric acid esters, including dimethyl fumarate (DMF, Tecfidera®) have shown therapeutic effects in several disease models, prompting us to determine whether DMF is effective as a treatment for SSc dermal fibrosis. We found that DMF blocks the pro-fibrotic effects of TGFβ in SSc skin fibroblasts. Mechanistically, we found that DMF treatment reduced nuclear localization of TAZ and YAP proteins via inhibition of PI3-K/Akt1 pathway...
September 1, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28726006/microrna-21-in-skin-fibrosis-potential-for-diagnosis-and-treatment
#6
REVIEW
Yan Li, Juan Zhang, Yuying Lei, Lechun Lyu, Ruiling Zuo, Ting Chen
Skin fibrosis is a common pathological process characterized by fibroblast proliferation and excessive deposition of extracellular matrix. However, the pathogenesis of the disease is still not clear. Previous studies have shown that microRNA-21 may play pivotal roles in the regulation of a variety of skin fibrosis, including keloid, scleroderma, and hypertrophic scar. In this review, we outline the structure, expression, and regulation of microRNA-21 and its role in fibrotic skin diseases. In future, it may be useful as a prognostic or diagnostic marker...
July 19, 2017: Molecular Diagnosis & Therapy
https://www.readbyqxmd.com/read/28712039/cutaneous-manifestations-of-scleroderma-and-scleroderma-like-disorders-a-comprehensive-review
#7
REVIEW
Caterina Ferreli, Giulia Gasparini, Aurora Parodi, Emanuele Cozzani, Franco Rongioletti, Laura Atzori
Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations...
July 16, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28703365/phototherapy-in-systemic-sclerosis-review
#8
REVIEW
Suteeraporn Chaowattanapanit, Charoen Choonhakarn, Chingching Foocharoen, Narachai Julanon
Systemic scleroderma-also known as systemic sclerosis (SSc)-is a chronic systemic connective tissue disease characterized by collagen deposition in cutaneous and internal organs, leading to skin sclerosis and multiple organ fibrosis. The pathogenesis is complex and remains poorly understood. Treatment is based on organ involvement and requires a multidisciplinary approach. Skin sclerosis can cause disability, leading to decreasing quality of life. Various systemic antifibrotic therapies have been used; however, most have unsatisfactory results...
July 13, 2017: Photodermatology, Photoimmunology & Photomedicine
https://www.readbyqxmd.com/read/28691464/parry-romberg-syndrome-and-linear-facial-scleroderma-management-in-pediatric-population
#9
E Segna, V Pucciarelli, G A Beltramini, C Sforza, F J Silvestre, A B Giannì, A Baj
Among all different types of cutaneous scleroderma, Parry Romberg syndrome and linear scleroderma “en coup de sabre” typically involve the visage. Gradual degeneration of the tissues, from the skin up to the bone, is the stigmata of the diseases and the range of clinical manifestations is wide. They typically start during childhood and slowly progress before stabilizing. Considering the gravity of the associated deformity and its impact on facial function and appearance, we strongly advocate a prompt intervention that however must be tailored on paediatric patients...
April 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28562527/successful-treatment-of-infliximab-in-a-patient-with-scleroderma-a-case-report
#10
Ting Li, Yaoyang Liu, Huji Xu
RATIONALE: Systemic Scleroderma (SSc) is a rare connective tissue disease clinically characterized by cutaneous sclerosis and variable systemic involvement. No drug is currently available to effectively reverse the fibrotic process in SSc. Previous reports have suggested that the tumor necrosis factor (TNF) antagonists could be useful for the treatment of fibrotic disorders. However, TNFα has long been considered as an antifibrotic cytokine. Whether TNF antagonist is effective for SSc patients needs to be tested...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28413064/systemic-sclerosis
#11
REVIEW
Christopher P Denton, Dinesh Khanna
Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis...
April 13, 2017: Lancet
https://www.readbyqxmd.com/read/28386330/geniposide-inhibited-endothelial-mesenchymal-transition-via-the-mtor-signaling-pathway-in-a-bleomycin-induced-scleroderma-mouse-model
#12
Qing Qi, Yueping Mao, Yongzhen Tian, Ke Zhu, Xushan Cha, Minghua Wu, Xiaodong Zhou
AIM: Geniposide is an iridoid glycoside isolated from the gardenia plant. It has multiple biological activities. The roles of geniposide in systemic sclerosis (SSc) and in endothelial-to-mesenchymal transition (EndMT) are unclear. We investigated the protective effects of geniposide in a bleomycin-induced SSc mouse model, and its potential mechanisms. METHODS: The effects of geniposide were evaluated as follows: (1) histological and immunochemical changes in mouse skin tissue; (2) changes in cellular morphology of human umbilical vein endothelial cells (HUVECs); (3) expression of endothelial cell biomarkers (E-Cadherin, CD31, and CD34), mesenchymal cell markers (FSP1, Collagen, and α-SMA), and key factors of EndMT (Slug, Snail, and Twist) using real time PCR, Western blot, and immunofluorescence; (4) tube formation in HUVECs; (5) mTOR signaling pathway transcription factors using Western blot analysis...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28376288/mycophenolate-mofetil-versus-placebo-for-systemic-sclerosis-related-interstitial-lung-disease-an-analysis-of-scleroderma-lung-studies-i-and-ii
#13
COMPARATIVE STUDY
Elizabeth R Volkmann, Donald P Tashkin, Ning Li, Michael D Roth, Dinesh Khanna, Anna-Maria Hoffmann-Vold, Grace Kim, Jonathan Goldin, Philip J Clements, Daniel E Furst, Robert M Elashoff
OBJECTIVE: To compare mycophenolate mofetil (MMF) with placebo for the treatment of systemic sclerosis (SSc)-related interstitial lung disease (ILD). METHODS: We included participants enrolled in the placebo arm of Scleroderma Lung Study (SLS) I and the MMF arm of SLS II. SLS I randomized participants to receive either oral cyclophosphamide (CYC) or placebo for 1 year, while SLS II randomized participants to receive either MMF for 2 years or oral CYC for 1 year followed by 1 year of placebo...
July 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28370513/high-dose-intravenous-immunoglobulin-therapy-for-scleromyxoedema-a-prospective-open-label-clinical-trial-using-an-objective-score-of-clinical-evaluation-system
#14
A Guarneri, M Cioni, F Rongioletti
BACKGROUND: Scleromyxoedema is a primary fibro-mucinosis whose therapy is still challenging. OBJECTIVE: To evaluate the safety and efficacy of high-dose intravenous immunoglobulin (IVIg) for the management of scleromyxoedema prospectively using an objective score. METHODS: In a prospective open-label study, IVIg was administered to eight patients with scleromyxoedema in a dose of 2 g/kg per month. The patients were followed-up to a minimum of 6 months, and their disease activity and response to treatment were assessed using the Physician's Global Assessment of disease severity (PGA) and a modified objective skin scoring system for patients with scleroderma (modified Rodnan score system for scleromyxoedema or mRSSS)...
March 29, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28349063/thermography-improves-clinical-assessment-in-patients-with-systemic-sclerosis-treated-with-ozone-therapy
#15
Danuta Nowicka
Objective. Treatment of scleroderma is challenging and limited. The aim of our study was to evaluate the usefulness of thermography in assessment of the clinical condition (joints movability and skin thickness) in clinically advanced patients with systemic sclerosis before and after ozone therapy. Method. The study included 42 patients aged 32 to 73 years with advanced systemic sclerosis hospitalized in the university clinic between 2003 and 2006. Thermography and clinical examinations were conducted at baseline and after two series of bath in water with ozone...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28337853/long-term-efficacy-and-tolerability-of-mycophenolate-mofetil-therapy-in-diffuse-scleroderma-skin-disease
#16
Daniel Boulos, Gene-Siew Ngian, Anton Rajadurai, Kathleen Elford, Wendy Stevens, Susanna Proudman, Claire Owen, Janet Roddy, Mandana Nikpour, Peter Youssef, Catherine Hill, Joanne Sahhar
OBJECTIVES: To assess the long-term efficacy and tolerability of mycophenolate mofetil (MMF) in patients with diffuse cutaneous systemic sclerosis (dcSSc). METHODS: Patients enrolled in the Australian Scleroderma Cohort study with dcSSc and baseline modified Rodnan skin score (mRSS) ≥ 12 who were treated for a minimum of 12 months with MMF for the primary indication of skin disease were included and their prospectively collected data retrieved. Change in mRSS, the proportion with a clinically significant improvement (reduction in mRSS ≥ 5 from baseline) and adverse effects due to therapy were determined...
April 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28303481/morphea-and-eosinophilic-fasciitis-an-update
#17
REVIEW
Jorre S Mertens, Marieke M B Seyger, Rogier M Thurlings, Timothy R D J Radstake, Elke M G J de Jong
Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated...
August 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28303284/-generalized-circumscribed-scleroderma-with-blisters
#18
G Wagner, V Meyer, M M Sachse
The patient suffered from a 20-year course of generalized circumscribed scleroderma and presented with blisters in circumscribed areas of the affected skin. The development of subepidermal blisters has been described in all clinical forms of circumscribed scleroderma. Aetiology and pathogenesis of blister formation have not yet been clarified. An obstruction of the lymphatic vessels due to the present sclerosis is favoured. Treatment of bullous circumscribed scleroderma is considered to be difficult. Oral steroids, methotrexate, hydroxychloroquine and PUVA methods have been used with varying success...
July 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28271552/localized-scleroderma-clinical-and-epidemiological-features-with-emphasis-on-adulthood-versus-childhood-onset-disease-differences
#19
REVIEW
A Lis-Święty, A Skrzypek-Salamon, I Ranosz-Janicka, L Brzezińska-Wcisło
BACKGROUND: Localized scleroderma is a rare inflammatory skin disorder that affects the skin and sometimes underlying subcutaneous tissue, muscles or bones. The disease has two modes of onset: juvenile- (JLS) and adult-onset (aLoS). Clinical features have impact on diagnostic and treatment recommendations, but no consensus on the disease management depending on the age at diagnosis was given. OBJECTIVE: To identify these features which differentiate aLoS from JLS...
March 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28263294/increased-expression-of-latent-tgf-%C3%AE-binding-protein-4-affects-the-fibrotic-process-in-scleroderma-by-tgf-%C3%AE-smad-signaling
#20
Jiaying Lu, Qingmei Liu, Lei Wang, Wenzhen Tu, Haiyan Chu, Weifeng Ding, Shuai Jiang, Yanyun Ma, Xiangguang Shi, Weilin Pu, Xiaodong Zhou, Li Jin, Jiucun Wang, Wenyu Wu
Scleroderma is a fibrosis-related disorder characterized by cutaneous and internal organ fibrosis, and excessive collagen deposition in extracellular matrix (ECM) is a major cause of fibrosis. Transforming growth factor-β (TGF-β)/SMAD signaling has a central role in the pathogenesis of fibrosis by inducing abnormal collagen accumulation in ECM, and latent TGF-β-binding protein 4 (LTBP-4) affects the secretion of latent TGF-β to ECM. A previous study indicated that bleomycin (BLM) treatment increased LTBP-4 expression in lung fibroblasts of Thy-1 knockout mice with lung fibrosis, and LTBP-4 further promoted TGF-β bioavailability as well as SMAD3 phosphorylation...
May 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
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