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Total 155 results found since Jan 2013.

Juvenile dermatomyositis: a tertiary center experience
This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003 –2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty. A total of 50 patients (35 females), median age at the onset 6.1 ± 4.1 years, were identified. Mean follow-up period was 74.5 ± 49.7 months. Presenting clinical symptoms included heliotrope rash (100%), Gottron papu le (96%), muscle weakness (90%), erythro...
Source: Clinical Rheumatology - January 4, 2017 Category: Rheumatology Source Type: research

Dermatomyositis Leading to Necrotizing Vasculitis: A Perfect Response to Applied Therapy.
Authors: Akbaryan M, Darabi F, Soltani Z Abstract Dermatomyositis is an idiopathic inflammatory myopathy that cause skin and muscle complications. The ethiology is not understood well yet. Released cytokines including interferon and interleukins are suggested to make inflammatory responses in the skin or muscle. Muscle weakness and skin lesions including heliotrope rash, shawl sign and Gottron's papules are the most common symptoms. A biopsy (muscle or skin) is always the most reliable method for diagnosis. Corticosteroids in association with immunosuppressive agents are used as standard treatment. The patient was ...
Source: International Journal of Biomedical Science - February 15, 2017 Category: Biomedical Science Tags: Int J Biomed Sci Source Type: research

RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis
Dermatomyositis (DM) is inflammatory myopathy or myositis characterized by muscle weakness and skin manifestations. In the differential diagnosis of DM the evaluation of the muscle biopsy is of importance amon...
Source: Arthritis Research and Therapy - July 24, 2017 Category: Rheumatology Authors: Xavier Su árez-Calvet, Eduard Gallardo, Iago Pinal-Fernandez, Noemi De Luna, Cinta Lleixà, Jordi Díaz-Manera, Ricardo Rojas-García, Ivan Castellví, M. Angeles Martínez, Josep M. Grau, Albert Selva-O’Callaghan and Isabel Illa Source Type: research

Dropped Head Syndrome and the Presence of Rimmed Vacuoles in a Muscle Biopsy in Scleroderma-Polymyositis Overlap Syndrome Associated with Anti-Ku Antibody.
Authors: Takeshi Y, Mai Y, Kinjo M, Manabu J, Itsuro H Abstract 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 my...
Source: Internal Medicine - November 21, 2017 Category: Internal Medicine Tags: Intern Med Source Type: research

Mutations in COA7 cause spinocerebellar ataxia with axonal neuropathy
AbstractSeveral genes related to mitochondrial functions have been identified as causative genes of neuropathy or ataxia. Cytochromec oxidase assembly factor 7 (COA7) may have a role in assembling mitochondrial respiratory chain complexes that function in oxidative phosphorylation. Here we identified four unrelated patients with recessive mutations inCOA7 among a Japanese case series of 1396 patients with Charcot-Marie-Tooth disease (CMT) or other inherited peripheral neuropathies, including complex forms of CMT. We also found that all four patients had characteristic neurological features of peripheral neuropathy and atax...
Source: Brain - April 27, 2018 Category: Neurology Source Type: research

Doctors Think a Woman ’s Tattoo May Have Been Causing Her Severe Leg Pain
Doctors in Scotland believe a woman’s large tattoo on her leg may have been the cause of her unexplained inflammation and severe leg pain. The authors of a report on the woman’s case, published in BMJ Case Reports, say it is a reminder to physicians that they should be mindful of their patients’ tattoos if they encounter unusual and unexplained symptoms. The 31-year-old woman was suffering a condition called inflammatory myopathy, which affected her left leg and caused pain severe enough to disrupt her sleep. The woman had a double lung transplant in 2009, and was taking drugs to suppress her immune syst...
Source: TIME: Health - June 19, 2018 Category: Consumer Health News Authors: Alice Park Tags: Uncategorized healthytime onetime tattoos Source Type: news

Fashionably Late: A Case of Delayed Cutaneous Manifestations in Juvenile Dermatomyositis.
Abstract Juvenile dermatomyositis (JDM) is a rare, but well recognized multi-systemic inflammatory myopathy in children defined by proximal muscle weakness and distinctive skin lesions, that if recognized and treated early result in decreased morbidity and mortality. The 1975 criteria established by Bohan and Peter center around the propensity for early development of heliotrope and Gottron's lesions in combination with specific laboratory abnormalities, and are still the leading diagnostic tool. The following case demonstrates a toddler with an atypical presentation of JDM in which delayed dermatologic manifestat...
Source: Clin Med Res - October 24, 2018 Category: Research Authors: Antoine M, Reeves PT, Rohena L, Jones O, Faux B Tags: J Clin Med Res Source Type: research

Cardiac Manifestations in Idiopathic Inflammatory Myopathies: An Overview
Idiopathic inflammatory myopathies are a group of autoimmune diseases that are characterized by muscle inflammation resulting in elevated muscle enzyme release and distinctive biopsy findings. This group of conditions includes polymyositis, dermatomyositis, inclusion body myositis, and necrotizing autoimmune myopathy. Although they have many similarities, the inflammatory myopathies differ in their clinical, pathological, and treatment realms. Extramuscular manifestations may involve many organs that include the skin, joints, heart, lungs, and gastrointestinal tract. Cardiovascular involvement is one of the leading causes ...
Source: Cardiology in Review - April 6, 2019 Category: Cardiology Tags: Review Articles Source Type: research

Neurological Involvement in Primary Systemic Vasculitis
Conclusion Neurological involvement is a common complication of PSV (Table 1), and neurologists play an important role in the identification and diagnosis of PSV patients with otherwise unexplained neurological symptoms as their chief complaint. This article summarizes the neurological manifestations of PSV and hopes to improve neuroscientists' understanding of this broad range of diseases. TABLE 1 Table 1. Common CNS and PNS involvements of primary systemic vasculitis. Author Contributions SZ conceived the article and wrote the manuscript. DY and GT reviewed and edited the manuscript. All authors ...
Source: Frontiers in Neurology - April 25, 2019 Category: Neurology Source Type: research

Systemic capillary leak syndrome in dermatomyositis: A report of 2 cases
Systemic capillary leak syndrome (SCLS) is a rare, often fatal, condition characterized by hemoconcentration, hypoalbuminemia and hypotension. Its association with other disease entities remains unclear. In this case report, we describe two diagnosed cases of dermatomyositis, an idiopathic inflammatory myopathy, complicated with SCLS. The first case is of a 41-year-old, male and the second case is of a 61-year-old female. Both cases were admitted for the management of dermatomyositis and presented with characteristic cutaneous lesions of the condition, proximal muscle weakness, elevated muscle enzymes, positive ANA, abnorm...
Source: Journal of the American Academy of Dermatology - August 21, 2019 Category: Dermatology Source Type: research

A Rare Case of Paralysis
​BY ALEXANDRA SALAS; JENNIFER TUONG; IVAN KHARCHENKO; VICTOR RIVERA; & AHMED RAZIUDDIN, MDA 27-year-old man with a past medical history of ADHD managed with Adderall presented to the emergency department with bilateral upper leg weakness associated with soreness since the day before. He had run 2.5 miles before his symptoms started.The symptoms progressively worsened until he was not able to walk or get up from a sitting position. He was also experiencing weakness in his arms. He had no associated trauma, headache, vision changes, chest pain, shortness of breath, fever, abdominal pain, nausea, vomiting, diarrhea, or ...
Source: The Case Files - August 27, 2019 Category: Emergency Medicine Tags: Blog Posts Source Type: research

EP.19Anti-HMGCR positive necrotizing myopathy presenting in childhood
A 12-year-old girl, previously healthy, was hospitalized because of progressive proximal weakness for a few months. CK was 17000U/L. There were only minor skin changes, but an initial muscle biopsy was reported to have changes possibly compatible with dermatomyositis. Treatment was started with prednisolone and methotrexate, without adequate improvement. A biopsy two years later did not exclude the diagnosis of a muscular dystrophy. The fast development of weakness did not however really fit clinically.
Source: Neuromuscular Disorders - September 30, 2019 Category: Neurology Authors: M. Rasmussen, A. Selvaag, S. Wallace, E. Kirkhus, E. Merckoll, E. Antal, J. Pahnke, B. Udd Source Type: research

Classical Dermatomyositis: A Case Report.
Authors: Pokhrel S, Pardhe BD, Giri N, Pokhrel R, Paudel D Abstract Dermatomyositis is an idiopathic myopathy involving progressive muscle weakness with skin manifestation. Diagnosis is based upon the progressive muscle weakness, skin rashes, elevated serum muscle enzymes, muscle biopsy, and abnormal electromyogram. The incidence of dermatomyositis is rare. In this case study, we report a case of classical dermatomyositis without incidence of malignancy. Timely diagnosis and administration of steroid led to better prognosis of the patient. PMID: 32104038 [PubMed]
Source: Clinical, Cosmetic and Investigational Dermatology - February 29, 2020 Category: Dermatology Tags: Clin Cosmet Investig Dermatol Source Type: research

Case Report: Calpainopathy Presenting After Bone Marrow Transplantation, With Studies of Donor Genetic Content in Various Tissue Types
This report emphasizes that genetic disease should still be considered in the context of presumably acquired disease, and also demonstrates the extent of transdifferentiation of donor cells into other tissues.
Source: Frontiers in Neurology - January 11, 2021 Category: Neurology Source Type: research

Begelomab for severe refractory dermatomyositis: A case report
Conclusion: Blockade of DPP-4/CD26, which is expressed on T cells and mediates T cell activation and function, is safe and might be effective in patients with refractory DM.
Source: Medicine - March 5, 2021 Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research