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scleroderma and skin and treatment

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https://www.readbyqxmd.com/read/29773274/scleroderma-in-children
#1
REVIEW
Francesco Zulian
Juvenile scleroderma with its two varieties, juvenile localized scleroderma and systemic sclerosis (SSc), represents the third most frequent rheumatic disease in childhood. In juvenile SSc, new developments have been recently reported in the fields of classification and monitoring. The introduction of new classification criteria for adult SSc has stimulated new ideas on how to improve the performance of the provisional 2007 PRES/ACR/EULAR pediatric criteria. The introduction of a multidimensional severity score, named "J4S," which includes parameters on growth, skin, and internal organ involvement, has improved the approach to the patients in the daily practice to guide decision-making...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29771006/the-anti-fibrotic-effect-of-adenosine-a2b-receptor-antagonism-in-a-mouse-model-of-dermal-fibrosis
#2
Harry Karmouty-Quintana, Jose G Molina, Kemly Philip, Chiara Bellocchi, Brent Gudenkauf, Minghua Wu, Ning-Yuan Chen, Scott D Collum, Junsuk Ko, Sandeep K Agarwal, Shervin Assassi, Hongyan Zhong, Michael R Blackburn, Tingting Weng
OBJECTIVE: Systemic sclerosis (SSc - scleroderma) is a chronic disease that affects the skin and various internal organs. Dermal fibrosis is a major component of this disease. The mechanisms that promote dermal fibrosis remain elusive. Elevations in tissue adenosine levels and the subsequent engagement of the pro-fibrotic A2B adenosine receptor (ADORA2B) have been shown to regulate fibrosis in multiple organs including the lung, kidney and penis; however, the role of the ADORA2B in dermal fibrosis has not been investigated...
May 17, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29760622/skin-and-mucous-membranes-manifestations-of-dermatological-diseases-within-the-genital-area-in-females
#3
Katarzyna Plagens-Rotman, Renata Przybylska, Zygmunt Adamski, Magdalena Czarnecka-Operacz
Introduction: Lichen sclerosus et atrophicus (LSA) and an inversed type of psoriasis belong to a group of benign dermatoses usually located within the region of female external genitalia. The most common subjective symptoms reported by patients are itching, pain and changes in the color and structure of the skin. Aim: This paper presents 3 cases of patients suffering from selected dermatoses located within the external female genitalia treated at the Department of Dermatology, Poznan University of Medical Sciences...
April 2018: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29729451/disease-course-and-long-term-outcome-of-juvenile-localized-scleroderma-experience-from-a-single-pediatric-rheumatology-centre-and-literature-review
#4
REVIEW
Giorgia Martini, Gloria Fadanelli, Anna Agazzi, Fabio Vittadello, Alessandra Meneghel, Francesco Zulian
Juvenile Localized Scleroderma (JLS) is a rare disorder that may cause severe aesthetic sequelae and functional disability. To date, data on natural history and long-term outcome are discordant and difficult to compare due to the heterogeneity of clinical subtypes, treatments and methods to evaluate activity and outcome in previous studies. A retrospective and cross-sectional study including 133 patients followed between January 1991 and December 2016 was conducted at our Pediatric Rheumatology Centre. Disease course was drawn by retrospective analysis of patients' clinical features, treatment, disease course and outcome at the last evaluation...
May 2, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29720235/effects-of-selexipag-and-its-active-metabolite-in-contrasting-the-profibrotic-myofibroblast-activity-in-cultured-scleroderma-skin-fibroblasts
#5
Maurizio Cutolo, Barbara Ruaro, Paola Montagna, Renata Brizzolara, Emanuela Stratta, Amelia Chiara Trombetta, Stefano Scabini, Pier Paolo Tavilla, Aurora Parodi, Claudio Corallo, Nicola Giordano, Sabrina Paolino, Carmen Pizzorni, Alberto Sulli, Vanessa Smith, Stefano Soldano
BACKGROUND: Myofibroblasts contribute to fibrosis through the overproduction of extracellular matrix (ECM) proteins, primarily type I collagen (COL-1) and fibronectin (FN), a process which is mediated in systemic sclerosis (SSc) by the activation of fibrogenic intracellular signaling transduction molecules, including extracellular signal-regulated kinases 1 and 2 (Erk1/2) and protein kinase B (Akt). Selexipag is a prostacyclin receptor agonist synthesized for the treatment of pulmonary arterial hypertension...
May 2, 2018: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29692255/juvenile-scleroderma-what-has-changed-in-the-meantime
#6
Amra Adrovic, Sezgin Sahin, Kenan Barut, Ozgur Kasapcopur
BACKGROUND: Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was reported as one per million. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. OBJECTIVES: Since it is uncommon in children, many aspects of the disease remain discussable. With this review, we aimed to revise recent findings and new developments in this rare condition...
April 22, 2018: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/29686439/clinical-pattern-of-systemic-sclerosis-in-central-ukraine-association-between-clinical-manifestations-of-systemic-sclerosis-and-hypertension
#7
Viktor Semenov, Olexandr Kuryata, Tatiana Lysunets
Objectives: Systemic sclerosis (SSc) is a rare disease of connective tissue, manifestations of which may vary in different geographical areas. We aimed to describe the clinical portrait of patients with SSc in Dnipropetrovsk region and to investigate how initial clinical and laboratory characteristics are connected with the presence of hypertension in SSc onset. Material and methods: Patients were enrolled to this study from the registry of SSc patients, established in the Rheumatology Department, Mechnikov Dnipropetrovsk Regional Clinic, Dnipro...
2018: Reumatologia
https://www.readbyqxmd.com/read/29660185/a-proposed-mechanism-for-central-centrifugal-cicatricial-alopecia
#8
Jacob Subash, Tiffany Alexander, Victoria Beamer, Amy McMichael
Central centrifugal cicatricial alopecia (CCCA) has an unknown mechanism. Analyzing other scarring diseases (lichen planopilaris, fibrotic kidney disease, and scleroderma) may help clarify the mechanism of scarring in CCCA. These diseases were chosen for comparison due to either their location of disease (skin or scalp specifically), or prominence in patients of African descent. Genetics, possible triggers, an autoimmune lymphocytic response, and epithelial to mesenchymal transition are potentially involved...
April 16, 2018: Experimental Dermatology
https://www.readbyqxmd.com/read/29655520/comparing-ultraviolet-light-a-photo-chemo-therapy-with-methotrexate-protocol-in-childhood-localized-scleroderma-evidence-from-systematic-review-and-meta-analysis-approach
#9
REVIEW
Edoardo Marrani, Ivan Foeldvari, Jordi Anton Lopez, Rolando Cimaz, Gabriele Simonini
OBJECTIVE: Localized scleroderma is a skin fibrosing disorder that, if untreated, may result in severe disability. The purpose of this systematic review is to compare the present evidence concerning the effectiveness of Methotrexate versus phototherapy, alone or associated with Psoralen, in childhood localized scleroderma. METHOD: A systematic search between January 1996 and May 2017 was performed to identify studies investigating the efficacy of Methotrexate (MTX) or phototherapy (UVA) for treating localized scleroderma with onset ≤18 years...
March 14, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29595279/-systemic-sclerosis-in-2017
#10
Tomáš Soukup, Tomáš Veleta
Systemic sclerosis is classed as a diffuse (systemic) disease of connective tissue. It is a heterogeneous disease significantly shortening life expectancy. Its etiology is unknown. Pathogenetic interplay is assumed to involve a triad of pathological autoimmune inflammation, vasculopathy and fibrosis. Clinical manifestations can be classed based on the preponderant pathogenetic process. Vasculopathy is manifested by secondary Raynauds phenomenon with abnormal findings on the nailfold capillaroscopy, skin telangiectasias, gastric antral vascular ectasia, life threatening scleroderma renal crisis, digital ulcerations and prognostically severe pulmonary arterial hypertension...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29536565/chronic-cutaneous-graft-versus-host-disease-in-children-a-report-of-14-patients-from-a-tertiary-care-pediatric-dermatology-clinic
#11
Arti Nanda, Maitham A A Husain, Waleed Al-Herz, Adla Almekaimi, Humoud Al-Sabah, Mohammad Al-Otaibi
BACKGROUND/OBJECTIVES: Allogeneic hematopoietic stem cell transplantation (HSCT) is a treatment option for many life-threatening disorders in children. Chronic graft-versus-host disease (cGVHD) is a significant complication of HSCT, and its treatment is challenging. Skin is the most common organ affected in cGVHD, with protean manifestations posing a challenge in diagnosis and management. The objective was to have a better understanding of the spectrum of chronic cutaneous GVHD (cc-GVHD) in children...
March 14, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29535035/inflammatory-arthritis-in-pediatric-patients-with-morphea
#12
Sakeen W Kashem, Colleen K Correll, Richard K Vehe, Patricia M Hobday, Bryce A Binstadt, Sheilagh M Maguiness
BACKGROUND: Morphea or localized scleroderma is an inflammatory disorder resulting in fibrosis of the skin and subcutaneous tissues. Joint contractures, arthralgias, and functional compromise are recognized associations of pediatric morphea. The co-existence of inflammatory arthritis and morphea is not well-described in the literature. OBJECTIVE: To investigate the relationship between pediatric morphea and inflammatory arthritis with regards to cutaneous, musculoskeletal, and laboratory findings and treatment regimens...
March 10, 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29534662/primary-bullous-keratopathy-in-a-patient-with-werner-syndrome-treated-with-corneal-transplant
#13
Divya Singh, Anita Ganger, Noopur Gupta, Murugesan Vanathi, Rajesh Khadgawat, Radhika Tandon
Here, we present, to the best of our knowledge, the first case of Werner syndrome with corneal blindness due to bilateral primary bullous keratopathy. Werner syndrome is a rare autosomal recessive disorder characterized by features of premature aging, insulin-dependent diabetes mellitus, osteoporosis, atherosclerosis, hypergonadotrophic hypogonadism, hypertriglyceridemia, scleroderma-like skin changes, and sarcomas. Among ocular manifestations, cataracts, cystoid macular edema, and retinal detachment have been reported...
March 9, 2018: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/29525847/ultrasound-characterization-of-cutaneous-ulcers-in-systemic-sclerosis
#14
Yossra A Suliman, Suzanne Kafaja, John Fitzgerald, Ximena Wortsman, Jonathan Grotts, Marco Matucci-Cerrinic, Veena K Ranganath, Daniel E Furst
Skin ulcers in scleroderma (SSc) patients are considered a major challenge, both in clinical assessment and treatment decisions. The objective of our study is to assess ultrasonographic (US) morphology of skin ulcers in SSc patients and evaluate if US will be of value in enhancing our clinical information and influence our management plans. We examined a convenience sample of 21 skin ulcers reported in 10 SSc patients by US. We used a previously published US definition of normal skin and developed a preliminary US definition of skin ulcer...
June 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29431303/eosinophilic-fasciitis-a-single-center-experience-of-seven-patients
#15
Oded Shamriz, Mariana Druker, Tzahi Neuman, Zvi Dranitzki, Yuval Tal
BACKGROUND: Eosinophilic fasciitis (EF) is a rare disease characterized by scleroderma-like skin, inflammation of deep muscle fascia, hypergammaglobulinemia, peripheral eosinophilia, and elevated erythrocyte sedimentation rate. OBJECTIVES: To present our experience in diagnosis and treatment of seven biopsy-proven EF patients in a large tertiary medical center. METHODS: We screened all patients who were admitted to our tertiary medical center and diagnosed with EF by tissue biopsies from January 2000 to January 2016...
February 2018: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29416936/characterization-and-treatment-of-unilateral-facial-muscle-spasm-in-linear-scleroderma-a-case-report
#16
Fabio Danisi, Emma Guidi
Background: Linear scleroderma has been associated with muscle spasms ipsilateral to skin lesions. Typically, spasms are located in trigeminal innervated muscles, leading to hemimasticatory spasm (HMS). Case Report: We report a case of linear scleroderma associated with spasm of muscles innervated not only by the trigeminal but also by the facial nerve. Discussion: We review the patient's successful treatment with incobotulinumtoxinA, a formulation of botulinum toxin that has not been reported for use in this condition...
2018: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/29408278/new-developments-on-skin-fibrosis-essential-signals-emanating-from-the-extracellular-matrix-for-the-control-of-myofibroblasts
#17
REVIEW
Jan-Niklas Schulz, Markus Plomann, Gerhard Sengle, Donald Gullberg, Thomas Krieg, Beate Eckes
Many different diseases are associated with fibrosis of the skin. The clinical symptoms can vary considerably with a broad range from isolated small areas to the involvement of the entire integument. Fibrosis is triggered by a multitude of different stimuli leading to activation of the immune and vascular system that then initiate fibroblast activation and formation of matrix depositing and remodeling myofibroblasts. Ultimately, myofibroblasts deposit excessive amounts of extracellular matrix with a pathological architecture and alterations in growth factor binding and biomechanical properties, which culminates in skin hardening and loss of mobility...
February 12, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29390268/case-report-one-case-of-primary-al-amyloidosis-repeatedly-misdiagnosed-as-scleroderma
#18
Linge Sun, Lei Zhang, Wenlu Hu, Tian-Fang Li, Shengyun Liu
INTRODUCTION: Amyloid light chain (AL) results from the deposition of immunoglobulin light chain fragments, and can affect multiple organs/systems. Our patient was diagnosed as scleroderma repeatedly because of extensive skin thickening and hardening, but the treatment was not effective. We did extensive laboratory examinations including serum/urine protein electrophoresis and flow cytometry assay of bone marrow aspiration. CONCLUSION: A diagnosis of primary AL amyloidosis was established...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29356051/optical-coherence-tomography-angiography-of-normal-skin-and-inflammatory-dermatologic-conditions
#19
Anthony J Deegan, Faezeh Talebi-Liasi, Shaozhen Song, Yuandong Li, Jingjiang Xu, Shaojie Men, Michi M Shinohara, Mary E Flowers, Stephanie J Lee, Ruikang K Wang
BACKGROUND: In clinical dermatology, the identification of subsurface vascular and structural features known to be associated with numerous cutaneous pathologies remains challenging without the use of invasive diagnostic tools. OBJECTIVE: To present an advanced optical coherence tomography angiography (OCTA) method to directly visualize capillary-level vascular and structural features within skin in vivo. METHODS: An advanced OCTA system with a 1310 nm wavelength was used to image the microvascular and structural features of various skin conditions...
March 2018: Lasers in Surgery and Medicine
https://www.readbyqxmd.com/read/29336866/regulatory-mechanisms-of-collagen-expression-by-interleukin-22-signaling-in-scleroderma-fibroblasts
#20
Soichiro Sawamura, Masatoshi Jinnin, Kuniko Inoue, Keitaro Yamane, Noritoshi Honda, Ikko Kajihara, Takamitsu Makino, Shinichi Masuguchi, Satoshi Fukushima, Hironobu Ihn
BACKGROUND: Various cytokines have been indicated to be involved in the pathogenesis of systemic sclerosis (SSc). IL-22 is one of the member of IL-10 cytokine family, and several studies have implicated IL-22 signaling in the pathogenesis of autoimmune diseases. OBJECTIVES: To clarify the role of IL-22 in the regulatory mechanism of ECM expression and to determine the contribution of IL-22 to the phenotype of SSc. METHODS: The effect of IL-22 on ECM expression in normal fibroblasts was determined by using PCR array, real-time PCR and immunoblotting...
April 2018: Journal of Dermatological Science
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