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scleroderma and skin and treatment

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...
October 10, 2016: Journal of Clinical Investigation
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...
September 20, 2016: Annales de Dermatologie et de Vénéréologie
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
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
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
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
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...
August 22, 2016: Bone Marrow Transplantation
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
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...
August 2, 2016: Annales de Dermatologie et de Vénéréologie
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
M Rovira, A Lopez-Guillermo, F Cervantes, E Montserrat, C Rozman
A patient is described who developed scleroderma one and a half years: after the diagnosis of idiopathic myelofibrosis (IM). In addition to suffering from the hematological features characteristic of the osteosclerotic phase of IM, the patient also developed Raynaud's phenomenon, marked distal sclerosis in the hands and painful ulcers on the fingertips, with physical findings consistent with scleroderma and positive anti-nuclear antibodies (fine-speckled pattern). The skin lesions showed a dramatic improvement after prednisone treatment but the patient eventually died of progressive myelofibrosis...
1990: Leukemia & Lymphoma
Marta Oliveira, Fabia Patinha, Antonio Marinho
Eosinophilic fasciitis is a systemic inflammatory disease characterized by symmetrical swelling and skin induration of the distal portions of the arms and/or legs, evolving into a scleroderma-like appearance, accompanied by peripheral blood eosinophilia. It is a rare disease with a poorly understood etiology. Corticosteroid treatment remains the standard therapy, either taken alone or in association with an immunosuppressive drug. This paper presents a case of a male patient with palpebral edema and marked eosinophilia, diagnosed with intestinal parasitic infection in October 2006...
2016: Reumatologia
Dinesh Khanna, Carlo Albera, Aryeh Fischer, Nader Khalidi, Ganesh Raghu, Lorinda Chung, Dan Chen, Elena Schiopu, Margit Tagliaferri, James R Seibold, Eduard Gorina
OBJECTIVE: Systemic sclerosis-associated interstitial lung disease (SSc-ILD) shares a number of clinical features and pathogenic mechanisms with idiopathic pulmonary fibrosis (IPF). This study was designed to evaluate the tolerability of the IPF treatment pirfenidone in SSc-ILD. The known gastrointestinal, skin, and liver adverse events (AE) of pirfenidone are of importance given the involvement of these organs in SSc. METHODS: All patients received pirfenidone and were randomized 1:1 to either a 2- or 4-week titration starting at 801 mg/day and finishing at a maintenance dose of 2403 mg/day...
September 2016: Journal of Rheumatology
Valentina Cestelli, Andreina Manfredi, Marco Sebastiani, Emanuela Praino, Francesca Cannarile, Dilia Giuggioli, Clodoveo Ferri
INTRODUCTION: Vascular involvement plays a decisive role in systemic sclerosis (SSc) pathogenesis; it is responsible for some important clinical manifestations of the disease such as Raynaud's phenomenon and digital ulcers (DU). Bosentan, a dual receptor endothelin antagonist, and iloprost, often in combination therapy, seems to be able to interfere with the scleroderma microangiopathy. OBJECTIVES: Aim of the study was to evaluate the effect of bosentan and iloprost on scleroderma microangiopathy, analyzed by means of capillaroscopic skin ulcer risk index (CSURI), in SSc patients treated for the prevention of DU...
June 16, 2016: Modern Rheumatology
Riccardo Papa, Paolo Nozza, Claudio Granata, Roberta Caorsi, Marco Gattorno, Alberto Martini, Paolo Picco
OBJECTIVES: Eosinophilic fasciitis is an uncommon scleroderma-like disorder characterised by induration and thickening of skin and soft tissue, usually associated with peripheral eosinophilia, poorly characterised in childhood. METHODS: We report 3 paediatric cases of eosinophilic fasciitis showing unusual clinical and histopathological features with a review of the literature. RESULTS: All cases presented progressive motility impairment started from upper limbs with no skin abnormalities...
May 2016: Clinical and Experimental Rheumatology
Jessica Sue Haber, Rodrigo Valdes-Rodriguez, Gil Yosipovitch
Chronic itch is a common skin manifestation in many connective tissue diseases. However, the cause of chronic itch in these diseases is still not fully understood. The complex, and in some cases incomplete understanding of, pathogenesis in each condition makes it difficult to target specific mediators of chronic itch. It is important to better understand the pathophysiology of chronic itch in these conditions, as this information might provide a fuller understanding of the pathogenesis of the diseases in general and lead to the development of more specific treatments for connective tissue diseases in patients with chronic itch...
June 3, 2016: American Journal of Clinical Dermatology
Nolwenn Sautereau, Aurélie Daumas, Romain Truillet, Elisabeth Jouve, Jéremy Magalon, Julie Veran, Dominique Casanova, Yves Frances, Guy Magalon, Brigitte Granel
BACKGROUND: Autologous adipose tissue injection is used in plastic surgery for correction of localized tissue atrophy and has also been successfully offered for treatment of localized scleroderma. We aimed to evaluate whether patients with systemic sclerosis (SSc) and facial handicap could also benefit from this therapy. METHODS: We included 14 patients (mean age of 53.8 ± 9.6 years) suffering from SSc with facial handicap defined by Mouth Handicap in Systemic Sclerosis Scale (MHISS) score more than or equal to 20, a Rodnan skin score on the face more than or equal to 1, and maximal mouth opening of less than 55 mm...
March 2016: Plastic and Reconstructive Surgery. Global Open
Padmanabha D Shenoy, Manish Bavaliya, Sujith Sashidharan, Kaveri Nalianda, Sreelakshmi Sreenath
BACKGROUND: Scleroderma is a systemic autoimmune disease characterized mainly by skin manifestations and involvement of various visceral organs, especially the lungs. Lung involvement is the leading cause of mortality in patients with scleroderma. There are data to suggest that cyclophosphamide (CYC) and mycophenolate mofetil (MMF) are effective in the management of scleroderma interstitial lung disease (SSc-ILD) but no head to head comparative data are available to date. METHODS: For the last 3 years, patients with SSc-ILD have been treated at our centre by protocol-based administration of intravenous CYC and MMF...
2016: Arthritis Research & Therapy
Kengo Akashi, Jun Saegusa, Sho Sendo, Keisuke Nishimura, Takuya Okano, Keiko Yagi, Masashi Yanagisawa, Noriaki Emoto, Akio Morinobu
BACKGROUND: Endothelin-1 (ET-1) is important in the pathogenesis of systemic sclerosis (SSc). ET-1 binds two receptors, endothelin type A (ETA) and endothelin type B (ETB). Dual ETA/ETB receptor antagonists and a selective ETA receptor antagonist are used clinically to treat SSc, and the effect of these antagonists on fibroblast activation has been described. However, the role of ETB receptor signaling in fibrogenesis is less clear. This study was conducted to evaluate the profibrotic function of ETB receptor signaling in a murine model of bleomycin (BLM)-induced scleroderma...
2016: Arthritis Research & Therapy
Dinesh Khanna, Christopher P Denton, Peter A Merkel, Thomas Krieg, Franck-Olivier Le Brun, Angelina Marr, Kelly Papadakis, Janet Pope, Marco Matucci-Cerinic, Daniel E Furst
IMPORTANCE: Digital ulcers in patients with systemic sclerosis are associated with pain and poor quality of life. Endothelin-1 promotes vasculopathy in systemic sclerosis after macitentan, an endothelin-1 blocker. OBJECTIVE: To evaluate the efficacy of macitentan in reducing the number of new digital ulcers in patients with systemic sclerosis. DESIGN, SETTING, AND PARTICIPANTS: Two international, randomized, double-blind, placebo-controlled trials (DUAL-1, DUAL-2) were conducted between January 2012 and February 2014...
May 10, 2016: JAMA: the Journal of the American Medical Association
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