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scleroderma and skin and treatment

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
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
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
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...
March 10, 2018: Clinical Rheumatology
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
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
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 2, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
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)
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...
January 22, 2018: Lasers in Surgery and Medicine
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...
December 29, 2017: Journal of Dermatological Science
Jung In Song, Silvanie Volz, Maria Eirini Liodaki, Peter Mailänder, Konstantinos Kalousis
OBJECTIVE: Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology, with heterogeneous clinical manifestations and chronic and often progressive course. The diffuse cutaneous form of SSc (dcSSc) is characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs. Patients with limited cutaneous SSc (lcSSc) generally have long-standing Raynaud's phenomenon before other manifestations of SSc appear. Over the last decade the Interest of adipose-derived cell therapy in regenerative medicine has increased continuously...
September 2017: Hellenic Journal of Nuclear Medicine
Marie Dominique Ah Kioon, Claudio Tripodo, David Fernandez, Kyriakos A Kirou, Robert F Spiera, Mary K Crow, Jessica K Gordon, Franck J Barrat
Systemic sclerosis (SSc) is a multisystem life-threatening fibrosing disorder that lacks effective treatment. The link between the inflammation observed in organs such as the skin and profibrotic mechanisms is not well understood. The plasmacytoid dendritic cell (pDC) is a key cell type mediating Toll-like receptor (TLR)-induced inflammation in autoimmune disease patients, including lupus and skin diseases with interface dermatitis. However, the role of pDCs in fibrosis is less clear. We show that pDCs infiltrate the skin of SSc patients and are chronically activated, leading to secretion of interferon-α (IFN-α) and CXCL4, which are both hallmarks of the disease...
January 10, 2018: Science Translational Medicine
Dhaval J Shah, Ram Hirpara, Corrie L Poelman, Adrianne Woods, Laura K Hummers, Fredrick M Wigley, Jean L Wright, Arti Parekh, Virginia D Steen, Robyn T Domsic, Ami A Shah
OBJECTIVES: We examined SSc patients with breast cancer to 1) identify the prevalence of radiation complications and 2) examine SSc outcomes in SSc patients who received radiation therapy (RT) as part of their cancer treatment. METHODS: Patients with SSc and breast cancer were identified from the Johns Hopkins and University of Pittsburgh Scleroderma Center databases. We examined whether erythema, blistering, ulceration, or thickening of the skin developed in the RT port...
January 5, 2018: Arthritis Care & Research
Keith M Sullivan, Ellen A Goldmuntz, Lynette Keyes-Elstein, Peter A McSweeney, Ashley Pinckney, Beverly Welch, Maureen D Mayes, Richard A Nash, Leslie J Crofford, Barry Eggleston, Sharon Castina, Linda M Griffith, Julia S Goldstein, Dennis Wallace, Oana Craciunescu, Dinesh Khanna, Rodney J Folz, Jonathan Goldin, E William St Clair, James R Seibold, Kristine Phillips, Shin Mineishi, Robert W Simms, Karen Ballen, Mark H Wener, George E Georges, Shelly Heimfeld, Chitra Hosing, Stephen Forman, Suzanne Kafaja, Richard M Silver, Leroy Griffing, Jan Storek, Sharon LeClercq, Richard Brasington, Mary E Csuka, Christopher Bredeson, Carolyn Keever-Taylor, Robyn T Domsic, M Bashar Kahaleh, Thomas Medsger, Daniel E Furst
BACKGROUND: Despite current therapies, diffuse cutaneous systemic sclerosis (scleroderma) often has a devastating outcome. We compared myeloablative CD34+ selected autologous hematopoietic stem-cell transplantation with immunosuppression by means of 12 monthly infusions of cyclophosphamide in patients with scleroderma. METHODS: We randomly assigned adults (18 to 69 years of age) with severe scleroderma to undergo myeloablative autologous stem-cell transplantation (36 participants) or to receive cyclophosphamide (39 participants)...
January 4, 2018: New England Journal of Medicine
Hayley Barnes, Anne E Holland, Glen P Westall, Nicole Sl Goh, Ian N Glaspole
BACKGROUND: Approximately one-third of individuals with interstitial lung disease (ILD) have associated connective tissue disease (CTD). The connective tissue disorders most commonly associated with ILD include scleroderma/systemic sclerosis (SSc), rheumatoid arthritis, polymyositis/dermatomyositis, and Sjögren's syndrome. Although many people with CTD-ILD do not develop progressive lung disease, a significant proportion do progress, leading to reduced physical function, decreased quality of life, and death...
January 3, 2018: Cochrane Database of Systematic Reviews
B Aranegui, J Jiménez-Reyes
Morphea is an inflammatory, fibrosing skin disorder. When it occurs in childhood, it is also known as localized juvenile scleroderma. It is more common in girls and typically appears around the age of 5 to 7 years. According to a recent classification system, morphea is divided into 5 types: circumscribed (plaque), linear, generalized, pansclerotic, and mixed. Approximately 40% of patients present extracutaneous manifestations. Childhood morphea is treated with phototherapy, oral or topical calcitriol, topical tacrolimus 0...
December 13, 2017: Actas Dermo-sifiliográficas
Obada Hasan, Muneeba Jessar, Muhammad Ashar, Shahryar Noordin, Tashfeen Ahmad
INTRODUCTION: Systemic sclerosis is a rare and progressive multisystem autoimmune disorder that is characterized pathologically by vascular abnormalities, connective tissue sclerosis and atrophy of skin and various internal organs (e.g., alimentary tract, lungs, heart, kidney, CNS), and autoantibodies. With an unknown etiology, Scleroderma is a complex polygenetic disease. A recent Genome Wide Association Study (GWAS) confirmed a strong association with the Major Histocompatibility Complex (MHC) and autoimmunity...
2018: International Journal of Surgery Case Reports
Laila Siddique, Hadjh Ahrns, Elizabeth Seiverling
Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis. While most cutaneous mastocytoses occur in children and are asymptomatic, TMEP occurs predominantly in adults and is associated with systemic manifestations, requiring medical management. TMEP is typically characterised by scattered red-brown macules on the trunk and extremities, but must be differentiated from other telangiectatic conditions such as scleroderma, hereditary haemorrhagic telangiectasia and telangiectasias secondary to cirrhosis...
November 30, 2017: BMJ Case Reports
Dilia Giuggioli, Andreina Manfredi, Federica Lumetti, Michele Colaci, Clodoveo Ferri
BACKGROUND: Skin ulcers (SU) are one of the most frequent manifestations of systemic sclerosis (SSc). SSc-SU are very painful, often persistent and recurrent; they may lead to marked impairment of patient's activities and quality of life. Despite their severe impact on the whole SSc patient's management, the proposed definition, classification criteria, and therapeutic strategies of SSc-SU are still controversial. OBJECTIVE: The present study aimed to elaborate a comprehensive proposal of definition, classification, and therapeutic strategy of SSc-SU on the basis of our long-term single center experience along with a careful revision of the world literature on the same topic...
February 2018: Autoimmunity Reviews
Yoshida Takeshi, Yoshida Mai, Mitsuyo Kinjo, Jonosono Manabu, Higuchi Itsuro
A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory myositis involving axial muscles may be seen either in inclusion body myositis or SSc-PM overlap syndrome...
November 20, 2017: Internal Medicine
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