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skin necrosis and dermatomyositis

Shigeaki Suzuki, Akinori Uruha, Norihiro Suzuki, Ichizo Nishino
Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve skeletal muscle as well as many other organs. The classification of inflammatory myopathies has been based on clinical diagnoses, pathological diagnoses, and autoantibodies, independently. The clinical phenotypes of inflammatory myopathies are characterized by various autoantibodies that are originally detected by RNA or protein immunoprecipitation. However, since the correlation between histological features and autoantibodies had not been fully elucidated, we created the "Integrated Diagnosis Project for Inflammatory Myopathies" in October 2010...
July 2017: Autoimmunity Reviews
Yale Liu, Lingling Peng, Liang Li, Chengfei Liu, Xiao Hu, Shengxiang Xiao, Yumin Xia
TWEAK participates in various cellular effects by engaging its receptor of Fn14. Increased levels of soluble TWEAK are associated with systemic autoimmunity in patients with lupus erythematosus, rheumatoid arthritis, or dermatomyositis. However, the role of TWEAK in bullous pemphigoid (BP) remains unknown. In this study, we found an elevated serum level of TWEAK and a positive correlation between serum TWEAK and anti-BP180 antibodies. Immunohistochemistry showed strong TWEAK and Fn14 expression and implied an opposite relationship between the TWEAK and BP180 expression in skin samples from BP patients...
July 2017: Journal of Investigative Dermatology
Rohit Aggarwal, Priyadarshini Loganathan, Diane Koontz, Zengbiao Qi, Ann M Reed, Chester V Oddis
OBJECTIVE: The aim was to assess the efficacy of rituximab for the cutaneous manifestations of adult DM and JDM. METHODS: Patients with refractory adult DM (n = 72) and JDM (n = 48) were treated with rituximab in a randomized placebo-phase-controlled trial [either rituximab early drug (week 0/1) or rituximab late arms (week 8/9), such that all subjects received study drug]. Stable concomitant therapy was allowed. Cutaneous disease activity was assessed using the Myositis Disease Activity Assessment Tool, which grades cutaneous disease activity on a visual analog scale...
February 2017: Rheumatology
L C Meng, Y Li, W Zhang, H J Hao, F Gao, Y Yuan
OBJECTIVE: To report the clinical and myopathological features of 16 patients with Jo-1 syndrome. METHODS: Sixteen patients were recruited in this study, who were diagnosed as Jo-1 syndrome in Department of Neurology of Peking University First Hospital from January, 2011 to July, 2015. The clinical data and myopathological data were analyzed. RESULTS: The mean onset age was 41±14 (21-68) years old. 87.5% was female. The median duration was 9...
August 2, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Rama Bandlamudi Atluri
Idiopathic inflammatory myopathies are relatively rare diseases. Polymyositis and dermatomyositis are more common in women than men (2:1 ratio), while inclusion body myositis is twice as common in men. Inflammatory myopathies are a heterogeneous group of chronic systemic autoimmune diseases with an annual incidence of two to five cases per million, characterized by muscle inflammation and progressive muscle weakness. There are three major diseases which includes Dermatomyositis (DM) including a distinct juvenile subtype (JDM), Polymyositis (PM), and Inclusion Body Myositis...
March 2016: Missouri Medicine
L Charbit, A-C Bursztejn, S Mohamed, P Kaminsky, B Lerondeau, A Barbaud, J Deibener-Kaminsky, J-L Schmutz
BACKGROUND: Dermatomyositis (DM) is an inflammatory disease associated with auto-antibodies in 50 to 70% of cases. A new antibody, anti MDA-5, has been described in association with a specific type of DM involving severe interstitial lung disease and minimal muscle disease. We report the first case of DM with MDA-5 antibodies and with interstitial lung disease and rapidly extensive digital necrosis. PATIENTS AND METHODS: A 28-year-old male was hospitalized for asthenia, myalgia and subacute dyspnea...
August 2016: Annales de Dermatologie et de Vénéréologie
B Volc-Platzer
Dermatomyositis is a rare idiopathic inflammatory myopathy that affects adults and children, mostly female. Hallmarks of the disease are myositis with necrosis, regeneration and perifascicular atrophy accompanied by a typical skin rash with heliotrope erythema, Gottron's sign, Gottron's papules and nail fold changes with splinter hemorrhage. Typical skin symptoms may appear 6 months up to 2 years before muscle involvement (amyopathic dermatomyositis). New myositis-specific antibodies may allow clinicoserologic correlations within a heterogeneous clinical spectrum...
August 2015: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Natalia Gallardo C, Omar Valenzuela L, Sebastián Ibáñez V
We report a 37 years old male with a dermatomyositis treated with oral cyclophosphamide. He was admitted to the hospital due to a zone of skin necrosis with purulent exudate, located in the second left toe. A complete blood count showed a leukocyte count of 2,600 cells/mm³. A Chest CAT scan showed a pneumomediastinum with emphysema of adjacent soft tissue. Cyclophosphamide was discontinued and leukocyte count improved. The affected toe was amputated and a chest CAT scan showed a partial resolution of the pneumomediastinum...
January 2015: Revista Médica de Chile
Anil Kumar B Patil, A T Prabhakar, Ajith Sivadasan, Mathew Alexander, Geeta Chacko
Necrotizing myopathy with pipestem capillaries is a form of chronic inflammatory myopathy, with histopathology showing necrotizing myopathy, minimal cellular infiltration, and microangiopathy. A 30-year-old female presented with progressive limb weakness of 6 months, with skin pigmentation and Raynaud's phenomenon. Serum creatine phosphokinase was 3990 u/L. Muscle biopsy showed necrotic fibers, focal sparse perivascular inflammation/perifascicular atrophy, endomysial/epimysial vessel wall thickening with luminal narrowing...
January 2015: Neurology India
D Di Rollo, D Abeni, M Tracanna, A Capo, P Amerio
The association between idiopathic inflammatory myopathy (IIM) and cancer has been extensively studied in adults. Many epidemiological studies demonstrated this association, which appears stronger for dermatomyositis (DM) than for polymyositis (PM). The first case suggesting an association between cancer and DM was reported in 1916. At present the reported incidence of cancer association with DM varies widely, from less than 7% to over 30%. Many early evidences came from case reports, but this association was later confirmed in case-control as well as in population-based studies...
October 2014: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
F Bellutti Enders, F van Wijk, R Scholman, M Hofer, B J Prakken, A van Royen-Kerkhof, W de Jager
OBJECTIVE: Juvenile dermatomyositis (DM) is a systemic autoimmune disorder of unknown immunopathogenesis in which the immune system targets the microvasculature of skeletal muscles, skin, and other organs. The current mainstay of therapy is a steroid regimen in combination with other immunosuppressive treatments. To date, no validated markers for monitoring disease activity have been identified, which hampers personalized treatment. This study was undertaken to identify a panel of proteins specifically related to active disease in juvenile DM...
August 2014: Arthritis & Rheumatology
C Fontaine, D Staumont-Sallé, P-Y Hatron, A Cotten, C Couturier
This review outlines the skin, vascular and musculoskeletal symptoms affecting the hand during systemic inflammatory diseases other than rheumatoid arthritis. Skin lesions are diagnosed clinically and their symptomatology is documented through an extensive series of photographs. These conditions may require specific care before a surgical procedure can be performed. Vascular lesions are also diagnosed clinically and their symptomatology is described in detail. It is important to recognize that acrocyanosis is always benign...
June 2014: Chirurgie de la Main
A Mimori, T Suzuki, N Satoh, H Nara, J Masuyama, T Yoshio, S Kano, S Minota
Abstract We report on five patients with dermatomyositis (DM) and cutaneous necrosis. Patients presented with classic DM skin eruptions, mild myositis, and a high incidence (4/5) of interstitial pneumonia. Cutaneous necrosis developed independently of steroid therapy, with the majority of lesions being cured following several months of sterilization treatment. In addition, one patient with accompanying cancer presented with multiple necrotic lesions. Topical treatment using gentiana violet against local infection was considered to have been essential in accelerating healing...
June 2000: Modern Rheumatology
Stephanie W Liu, Nicole F Velez, Christina Lam, Alisa Femia, Scott R Granter, Henry B Townsend, Ruth Ann Vleugels
IMPORTANCE: Biologic therapies, including anti-tumor necrosis factor (TNF) agents, are increasingly used to treat a variety of autoimmune diseases. Paradoxically, these agents have been reported to induce some of the very diseases they were designed to treat, including dermatomyositis (DM). We describe the first case of anti-TNF-associated DM without muscle involvement presenting in an adult patient with a history of arthritis since childhood. This cutaneous eruption recurred after rechallenge with an alternate anti-TNF agent...
October 2013: JAMA Dermatology
J Wang, G Guo, G Chen, B Wu, L Lu, L Bao
BACKGROUND: Although some features of dermatomyositis (DM) and polymyositis (PM) have been reported as possible prognostic indicators for cancer development, previous studies were small in size and were unable to establish a definitive relationship between neoplasms and DM and PM. OBJECTIVES: To evaluate risk factors for developing malignancies in patients with DM and PM. METHODS: Meta-analysis of the studies reported in the literature was performed to unveil risk factors for developing cancer among patients with DM and PM...
October 2013: British Journal of Dermatology
Branka Marinović, Ines Lakos Jukić, Jasna Lipozencić
Autoimmune bullous dermatoses are a group of skin and/or mucous membrane diseases characterized by blisters and erosions, which are the results of autoantibodies directed to structural components of desmosomes and structural proteins of the basement membrane zone. In this group of diseases, the diagnosis is based on history, clinical presentation, histopathologic findings, findings of direct and indirect immunofluorescence, and specific evidence of circulating antibodies by the enzyme linked immunosorbent assay (ELISA) method...
October 2012: Acta Medica Croatica: C̆asopis Hravatske Akademije Medicinskih Znanosti
Julia Yu-Yun Lee, Chao-Kai Hsu, Ming-Fei Liu, Sheau-Chiou Chao
OBJECTIVE: Persistent pruritic eruptions (PPE) are common among our patients with adult-onset Still disease (AOSD). We aimed to characterize the clinicopathologic features of the AOSD-associated evanescent and persistent rashes. METHODS: We reviewed the clinicopathologic features of the skin lesions from all AOSD cases diagnosed in our hospital during 1988 to 2009. The diagnoses were based on Yamaguchi criteria for AOSD. RESULTS: Altogether, there were 36 patients (6 men and 30 women) with age of onset ranging from 17 to 67 years (average 35...
December 2012: Seminars in Arthritis and Rheumatism
Adam S Nabatian, Muhammad M Bashir, Maria Wysocka, Meena Sharma, Victoria P Werth
INTRODUCTION: Several studies have reported that TNFα is substantially increased within skin lesions of patients with discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE) and dermatomyositis (DM) compared to controls. Elevated TNFα has been reported in the sera of some patients with systemic lupus erythematosus, DLE and SCLE, but not in the sera of patients with DM. Because of the key pathogenic role of autoimmunity in these diseases, in this study we sought to evaluate TNFα production by a readily available source of immune cells (namely, peripheral blood mononuclear cells (PBMCs)) taken from controls and from patients with cutaneous lupus or DM...
2012: Arthritis Research & Therapy
Noah Samuels, Yaakov H Applbaum, Yaacov Esayag
An 81-year-old woman with adenocarcinoma of the rectosigmoid presented with progressive muscle weakness and difficulty swallowing, with symptoms worsening following successful resection of the tumor. On examination, she had weakness primarily of lower limb proximal muscles, with no other abnormal findings. Laboratory tests showed significant elevation of creatine kinase, and EMG findings indicated myositis of the proximal muscles. While MRI showed increased signal intensity on T2-weighted images of the leg muscles, indicating inflammation, muscle biopsy found widespread necrosis with only weak and focal lymphocytic infiltration...
June 2013: Rheumatology International
Jin-Kyoung Park, Han-Gyul Yoo, Dae-Seon Ahn, Hyun-Soon Jeon, Wan-Hee Yoo
Dermatomyositis (DM) is a systemic autoimmune disorder characterized by the inflammation of skeletal muscles and pathognomonic skin rashes, namely heliotrope rash and Gottron's papules and involvement of other organs. Interstitial lung disease (ILD) seems to be one of the most characteristic manifestations of the lung and associated with increased morbidity and mortality in patients with DM. Despite DM-associated ILD requires aggressive therapy with cytotoxic agents, the efficacy is questionable in some cases, and more effective and less toxic therapies are needed...
November 2012: Rheumatology International
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