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scleroderma and skin and treatment

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https://www.readbyqxmd.com/read/28210455/morphea-en-coup-de-sabre-an-unusual-oral-presentation
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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-beta (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 have previously identified CD109 as a TGF- β co-receptor and inhibitor of TGF-ß 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...
November 17, 2016: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27824545/targeted-therapy-in-systemic-sclerosis
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/27638441/phototherapy-for-sclerosing-skin-conditions
#13
Noelle M Teske, Heidi T Jacobe
Phototherapy is an effective treatment strategy for a variety of sclerosing skin conditions. There are a number of phototherapeutic modalities used for the treatment of sclerosing skin conditions, including ultraviolet (UV)A1, broadband UVA, psoralen plus UVA, and narrowband UVB phototherapy. As controlled trials with validated outcome measures are lacking for these therapies, existing evidence is largely level II for morphea and is even more minimal for scleroderma and other sclerosing disorders (scleroderma, lichen sclerosus, and chronic graft-versus-host disease, among others)...
September 2016: Clinics in Dermatology
https://www.readbyqxmd.com/read/27604889/localized-scleroderma-a-clinical-review
#14
Kirk Sperber, Julia Ash, Farrah Gutwein, Amy Wasserrman, Varuni Rao, Mark Tratenberg
Localized scleroderma (LS) is characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissue or both. The outcome for most patients with localized scleroderma depends upon the type and extent of the lesion. The major challenge for untreated patients is not survival but morbidity due to skin, muscle and bone atrophy resulting in growth defects. Treatment is individualized to type and stage of the lesion and may include pharmacologic and non-pharmacologic therapies. Among the pharmacologic modalities, methotrexate with systemic glucocorticoids is currently the mainstay of treatment...
September 7, 2016: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/27578917/scleroderma-renal-crisis-during-intravenous-cyclophosphamide-pulse-therapy-for-complicated-interstitial-lung-disease-was-successfully-treated-with-angiotensin-converting-enzyme-inhibitor-and-plasma-exchange
#15
Norihiro Nagamura, Seikon Kin
Systemic sclerosis (SSc) is a multiorgan disorder involving the skin, heart, lungs, kidneys, and intestines. Progressive interstitial lung disease (ILD) is a serious complication in SSc patients, and cyclophosphamide (CYC) is the only recommended therapy for this condition;(1)) however, its clinical effectiveness is not sufficient. Scleroderma renal crisis (SRC) is a rare complication, characterized by acute renal failure and progressive hypertension. Angiotensin-converting-enzyme inhibitor (ACE-i) is a widely accepted therapy for SRC...
August 2016: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/27578072/itch-management-physical-approaches-uv-phototherapy-acupuncture
#16
Isaac H Y Chan, Dedee F Murrell
BACKGROUND: Physical therapies refer to non-medical treatment strategies, including surgery, cryotherapy, UV phototherapy, and acupuncture. Most physical approaches are inappropriate in the context of itch. UV phototherapy and acupuncture may be effective in the management of itch. METHODS: A literature search was performed using MEDLINE and EMBASE. Bibliographies were reviewed for relevant articles. RESULTS: Narrowband UVB (311-313 nm) and UVA1 (340-400 nm) are equally effective in managing atopic dermatitis and associated itch...
2016: Current Problems in Dermatology
https://www.readbyqxmd.com/read/27548467/autologous-hematopoietic-stem-cell-transplantation-has-better-outcomes-than-conventional-therapies-in-patients-with-rapidly-progressive-systemic-sclerosis
#17
N Del Papa, F Onida, E Zaccara, G Saporiti, W Maglione, E Tagliaferri, R Andracco, D Vincenti, T Montemurro, L Mircoli, C Vitali, A Cortelezzi
We retrospectively evaluated the efficacy of autologous hematopoietic stem cell transplantation (AHSCT) in 18 patients with rapidly progressive diffuse cutaneous systemic sclerosis (rp-dcSSc), and compared their disease outcomes with those of 36 demographically- and clinically-matched patients treated with conventional therapies. Cutaneous involvement, by performing modified Rodnan skin score (mRss), lung diffusion capacity, by measuring diffusing capacity of lung for carbon monoxide (DLCO), and disease activity, by applying the European Scleroderma Study Group (ESSG) scoring system, were the outcome variables measured at the baseline time and then every 12 months for the following 60 months in both the AHSCT-treated patients and the control group...
January 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27510285/fractional-carbon-dioxide-laser-versus-low-dose-uva-1-phototherapy-for-treatment-of-localized-scleroderma-%C3%A2-a-clinical-and-immunohistochemical-randomized-controlled-study
#18
RANDOMIZED CONTROLLED TRIAL
S M Shalaby, M Bosseila, M M Fawzy, D M Abdel Halim, S S Sayed, R S H M Allam
Morphea is a rare fibrosing skin disorder that occurs as a result of abnormal homogenized collagen synthesis. Fractional ablative laser resurfacing has been used effectively in scar treatment via abnormal collagen degradation and induction of healthy collagen synthesis. Therefore, fractional ablative laser can provide an effective modality in treatment of morphea. The study aimed at evaluating the efficacy of fractional carbon dioxide laser as a new modality for the treatment of localized scleroderma and to compare its results with the well-established method of UVA-1 phototherapy...
November 2016: Lasers in Medical Science
https://www.readbyqxmd.com/read/27496274/-pansclerotic-morphea-with-a-rapidly-fatal-outcome-in-an-11-year-old-girl
#19
J Hardy, C Audouin-Pajot, A Abid, D Chiotasso, B Coustets, A Suc, P Timsit, L Mouthon, J Mazereeuw-Hautier
BACKGROUND: Pansclerotic morphea is a poorly described but extremely debilitating variant of localized scleroderma. We report a case with a rapidly fatal outcome in an 11-year-old girl. PATIENTS AND METHODS: An 11-year-old girl with a 2-year history of morphea presented at our institution in April 2012. The sclerosis had started on her trunk and progressed rapidly to involve her entire skin. Initial treatment with corticosteroids was ineffective and she presented extremely painful ulcerations of the lower limbs...
December 2016: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/27493845/scleroderma-in-a-patient-on-capecitabine-is-this-a-variant-of-hand-foot-syndrome
#20
Muhammad W Saif, Archana Agarwal, James Hellinger, Dorothy J Park, Elizabeth Volkmann
Drug-induced scleroderma is a rare adverse effect of some chemotherapeutic drugs, such as taxanes and bleomycin. Capecitabine, an oral fluoropyrimidine approved for the treatment of metastatic breast and colon cancer, commonly causes cutaneous side effects including the hand-and-foot syndrome (HFS). Scleroderma-like skin changes associated with HFS associated with capecitabine is rare. However, diffuse scleroderma has never before been reported. We report a case of capecitabine-induced diffuse/systemic scleroderma in an 86-year-old female treated with capecitabine for metastatic colorectal cancer...
2016: Curēus
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