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scleroderma and skin and treatment

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https://www.readbyqxmd.com/read/28337853/long-term-efficacy-and-tolerability-of-mycophenolate-mofetil-therapy-in-diffuse-scleroderma-skin-disease
#1
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...
March 24, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28303481/morphea-and-eosinophilic-fasciitis-an-update
#2
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...
March 16, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28303284/-generalized-circumscribed-scleroderma-with-blisters
#3
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...
March 16, 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
#4
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
#5
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...
March 6, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/28261202/stretching-reduces-skin-thickness-and-improves-subcutaneous-tissue-mobility-in-a-murine-model-of-systemic-sclerosis
#6
Ying Xiong, Lisbeth Berrueta, Katia Urso, Sara Olenich, Igla Muskaj, Gary J Badger, Antonios Aliprantis, Robert Lafyatis, Helene M Langevin
OBJECTIVE: Although physical therapy can help preserve mobility in patients with systemic sclerosis (SSc), stretching has not been used systematically as a treatment to prevent or reverse the disease process. We previously showed in rodent models that stretching promotes the resolution of connective tissue inflammation and reduces new collagen formation after injury. Here, we tested the hypothesis that stretching would impact scleroderma development using a mouse sclerodermatous graft-versus-host disease (sclGvHD) model...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28254742/pirfenidone-ameliorates-murine-chronic-gvhd-through-inhibition-of-macrophage-infiltration-and-tgf-%C3%AE-production
#7
Jing Du, Katelyn Paz, Ryan Flynn, Ante Vulic, Tara M Robinson, Katie E Lineburg, Kylie A Alexander, Jingjing Meng, Sabita Roy, Angela Panoskaltsis-Mortari, Michael Loschi, Geoffrey R Hill, Jonathan S Serody, Ivan Maillard, David Miklos, John Koreth, Corey S Cutler, Joseph H Antin, Jerome Ritz, Kelli P MacDonald, Timothy W Schacker, Leo Luznik, Bruce R Blazar
Allogeneic hematopoietic stem cell transplantation is hampered by chronic graft-versus-host disease (cGVHD) resulting in multi-organ fibrosis and diminished function. Fibrosis in lung and skin leads to progressive bronchiolitis obliterans (BO) and scleroderma, respectively, for which new treatments are needed. We evaluated pirfenidone, a FDA approved drug for idiopathic pulmonary fibrosis, for its therapeutic effect in cGVHD mouse models with distinct pathophysiology. In a full MHC-mismatched, multi-organ system model with BO, donor T cell responses that support pathogenic antibody production are required for cGVHD development...
March 2, 2017: Blood
https://www.readbyqxmd.com/read/28249987/trpv4-ion-channel-is-a-novel-regulator-of-dermal-myofibroblast-differentiation
#8
Shweta Sharma, Rishov Goswami, Michael Merth, Jonathan Cohen, Kai Y Lei, David X Zhang, Shaik O Rahaman
Scleroderma is a multisystem fibroproliferative disease with no effective medical treatment. Myofibroblasts are critical to the fibrogenic tissue repair process in the skin and many internal organs. Emerging data support a role for both matrix stiffness, and transforming growth factor β1 (TGFβ1), in myofibroblast differentiation. Transient receptor potential vanilloid 4 (TRPV4) is a mechanosensitive ion channel activated by both mechanical and biochemical stimuli. The objective of this study was to determine the role of TRPV4 in TGFβ1- and matrix stiffness-induced differentiation of dermal fibroblasts...
March 1, 2017: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/28210455/morphea-en-coup-de-sabre-an-unusual-oral-presentation
#9
Sven Niklander, Constanza Marín, René Martínez, Alfredo Esguep
Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival recession and alveolar bone resorption. We report a case of a 13-year-old girl who consulted for progressive recession on the attached gingiva of her upper left incisors. She also presented a hypopigmented line on the left side skin of her upper lip, which continued through the vermilion and the lip mucosa, including the gingiva of the affected teeth...
February 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/28188239/treatment-outcome-in-early-diffuse-cutaneous-systemic-sclerosis-the-european-scleroderma-observational-study-esos
#10
Ariane L Herrick, Xiaoyan Pan, Sébastien Peytrignet, Mark Lunt, Roger Hesselstrand, Luc Mouthon, Alan Silman, Edith Brown, László Czirják, Jörg H W Distler, Oliver Distler, Kim Fligelstone, William J Gregory, Rachel Ochiel, Madelon Vonk, Codrina Ancuţa, Voon H Ong, Dominique Farge, Marie Hudson, Marco Matucci-Cerinic, Alexandra Balbir-Gurman, Øyvind Midtvedt, Alison C Jordan, Paresh Jobanputra, Wendy Stevens, Pia Moinzadeh, Frances C Hall, Christian Agard, Marina E Anderson, Elisabeth Diot, Rajan Madhok, Mohammed Akil, Maya H Buch, Lorinda Chung, Nemanja Damjanov, Harsha Gunawardena, Peter Lanyon, Yasmeen Ahmad, Kuntal Chakravarty, Søren Jacobsen, Alexander J MacGregor, Neil McHugh, Ulf Müller-Ladner, Gabriela Riemekasten, Michael Becker, Janet Roddy, Patricia E Carreira, Anne Laure Fauchais, Eric Hachulla, Jennifer Hamilton, Murat İnanç, John S McLaren, Jacob M van Laar, Sanjay Pathare, Susannah Proudman, Anna Rudin, Joanne Sahhar, Brigitte Coppere, Christine Serratrice, Tom Sheeran, Douglas J Veale, Claire Grange, Georges-Selim Trad, Christopher P Denton
OBJECTIVES: The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches. METHODS: This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening). Clinicians selected one of four protocols for each patient: methotrexate, mycophenolate mofetil (MMF), cyclophosphamide or 'no immunosuppressant'...
February 10, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28138238/quantitative-nanohistological-investigation-of-scleroderma-an-atomic-force-microscopy-based-approach-to-disease-characterization
#11
Adam P Strange, Sebastian Aguayo, Tarek Ahmed, Nicola Mordan, Richard Stratton, Stephen R Porter, Susan Parekh, Laurent Bozec
Scleroderma (or systemic sclerosis, SSc) is a disease caused by excess crosslinking of collagen. The skin stiffens and becomes painful, while internally, organ function can be compromised by the less elastic collagen. Diagnosis of SSc is often only possible in advanced cases by which treatment time is limited. A more detailed analysis of SSc may provide better future treatment options and information of disease progression. Recently, the histological stain picrosirius red showing collagen register has been combined with atomic force microscopy (AFM) to study SSc...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28116278/calcinosis-cutis-circumscripta-of-knee-a-rare-presentation
#12
Vikram V Kadu, K A Saindane, Ninad Godghate, Neha Godghate
INTRODUCTION: Calcinosis circumscripta is a form of ectopic mineralization characterized by single or multiple cutaneous nodules containing calcium salts. Calcinosis cutis circumscripta is a very rare form of idiopathic calcinosis cutis arising in the second half oflife. It typically involves the extremities and is associated with prior trauma and scleroderma. We dealt with a very rare form of calcinosis cutis circumscripta in a healthy patient, for whom surgical excision revealed to be an effective and successful treatment...
July 2016: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28107865/adipose-tissue-derived-stem-cells-ameliorates-dermal-fibrosis-in-a-mouse-model-of-scleroderma
#13
Wei Chen, Zhi-Kuan Xia, Man-Hui Zhang, Gui-Chun Ding, Xiao-Yan Zhang, Zheng-Xu Wang, Rong-Ya Yang
OBJECTIVE: To investigate the therapeutic potential of adipose-derived stem cells (ADSCs) for limited cutaneous scleroderma (LS) in mouse models. METHODS: ADSCs were isolated from pathogen-free female C57BL/6 mice and LS was induced in wild type (WT) C57BL/6 mice via daily injection of bleomycin (0.1 mL × 300 μg/mL) for 4 weeks; then the ADSCs were subcutaneously injected into the dorsal area in the model treatment group, and 100 μL of phosphate-buffered saline (PBS) solution was injected into the same site in the model control group...
January 2017: Asian Pacific Journal of Tropical Medicine
https://www.readbyqxmd.com/read/28065485/systemic-sclerosis-sine-scleroderma-in-mexican-patients-case-reports
#14
Olga Vera-Lastra, Christian Alexis Sauceda-Casas, María Del Pilar Cruz Domínguez, Sergio Alberto Mendoza Alvarez, Jesús Sepulceda-Delgado
Systemic sclerosis sine scleroderma (ssSSc) is a form of systemic sclerosis that is characterized by Raynaud's phenomenon (RP), visceral involvement without thickening of skin and anticentromere antibodies (ACA). We studied 10 ssSsc patients with a prevalence of 2%. The clinical signs were: RP 9/10, esophageal manifestations 8/10, pulmonary arterial hypertension 4/10, interstitial lung disease 4/10, cardiac signs 3/10 and ACA 8/10. CONCLUSION: In patients with RP, esophageal dysmotility, interstitial lung disease and pulmonary arterial hypertension should be tested for ACA in order to establish a prompt diagnosis and treatment of ssSSc...
January 3, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/27939457/skin-fibrosis-models-and-mechanisms
#15
N N Do, S A Eming
Matrix synthesis, deposition and remodeling are complex biological processes that are critical in development, maintenance of tissue homeostasis and repair of injured tissues. Disturbances in the regulation of these processes can result in severe pathological conditions which are associated with tissue fibrosis as e.g. in Scleroderma, cutaneous Graft-versus-Host-Disease, excessive scarring after trauma or carcinogenesis. Therefore, finding efficient treatments to limit skin fibrosis is of major clinical importance...
October 2016: Current Research in Translational Medicine
https://www.readbyqxmd.com/read/27866969/overexpression-of-cd109-in-the-epidermis-differentially-regulates-alk1-versus-alk5-signaling-and-modulates-extracellular-matrix-synthesis-in-the-skin
#16
Joshua Vorstenbosch, Christopher M Nguyen, Shufeng Zhou, You Jung Seo, Aya Siblini, Kenneth W Finnson, Albane A Bizet, Simon D Tran, Anie Philip
Transforming growth factor-β (TGF-β) is a multifunctional growth factor involved in many physiological processes including wound healing and inflammation. Excessive TGF-β signaling in the skin has been implicated in fibrotic skin disorders such as keloids and scleroderma. We previously identified CD109 as a TGF-β co-receptor and inhibitor of TGF-β signaling and have shown that transgenic mice overexpressing CD109 in the epidermis display decreased scarring. In certain cell types, in addition to the canonical type I receptor, ALK5, which activates Smad2/3, TGF-β can signal through another type I receptor, ALK1, which activates Smad1/5...
March 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27824545/targeted-therapy-in-systemic-sclerosis
#17
REVIEW
Murray Baron
Targeted therapies use an understanding of the pathophysiology of a disease in an individual patient. Although targeted therapy for systemic sclerosis (SSc, scleroderma) has not yet reached the level of patient-specific treatments, recent developments in the understanding of the global pathophysiology of the disease have led to new treatments based on the cells and pathways that have been shown to be involved in the disease pathogenesis. The presence of a B cell signature in skin biopsies has led to the trial of rituximab, an anti-CD20 antibody, in SSc...
October 31, 2016: Rambam Maimonides Medical Journal
https://www.readbyqxmd.com/read/27796521/long-term-clinical-stabilization-of-scleroderma-patients-treated-with-a-chronic-and-intensive-iv-iloprost-regimen
#18
Rosario Foti, Elisa Visalli, Giorgio Amato, Alessia Benenati, Giovanni Converso, Alberto Farina, Salvatore Bellofiore, Massimiliano Mulè, Marcella Di Gangi
Intravenous iloprost is a first-line option for the treatment of scleroderma-related digital vasculopathy, and some studies have suggested its favourable role on disease progression. The aim of our study is to evaluate the disease progression, specifically in terms of cardiopulmonary function, in a group of consecutive patients chronically treated with intravenous iloprost. Our retrospective study enrolled 68 scleroderma patients (68 F, 54.4 ± 12.3 years) treated with iloprost for 7.1 ± 2.9 years, with a schedule of 5-6 consecutive daily infusions per month (6 h/day, 0...
February 2017: Rheumatology International
https://www.readbyqxmd.com/read/27721238/dendritic-cells-maintain-dermal-adipose-derived-stromal-cells-in-skin-fibrosis
#19
Jennifer J Chia, Tong Zhu, Susan Chyou, Dragos C Dasoveanu, Camila Carballo, Sha Tian, Cynthia M Magro, Scott Rodeo, Robert F Spiera, Nancy H Ruddle, Timothy E McGraw, Jeffrey L Browning, Robert Lafyatis, Jessica K Gordon, Theresa T Lu
Scleroderma is a group of skin-fibrosing diseases for which there are no effective treatments. A feature of the skin fibrosis typical of scleroderma is atrophy of the dermal white adipose tissue (DWAT). Adipose tissue contains adipose-derived mesenchymal stromal cells (ADSCs) that have regenerative and reparative functions; however, whether DWAT atrophy in fibrosis is accompanied by ADSC loss is poorly understood, as are the mechanisms that might maintain ADSC survival in fibrotic skin. Here, we have shown that DWAT ADSC numbers were reduced, likely because of cell death, in 2 murine models of scleroderma skin fibrosis...
November 1, 2016: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27663385/-facial-linear-scleroderma-associated-with-neurological-abnormalities-relating-to-microangiopathy
#20
L Legendre, L Cuinat, J Curot, F Tanchoux, F Bonneville, J Mazereeuw-Hautier
BACKGROUND: Linear scleroderma is a fibrotic disease affecting the skin and sometimes the deeper tissues. We describe a case of scleroderma associated with neurological anomalies not previously reported in the literature. PATIENTS AND METHODS: A 16-year-old male patient presented in 2009 for hemifacial linear scleroderma. Treatment with methotrexate for 14 months resulted in stabilization of the disease. In 2013, we noted worsening of the patient's skin lesions as well as homolateral ptosis...
December 2016: Annales de Dermatologie et de Vénéréologie
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