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https://www.readbyqxmd.com/read/29732705/persistent-acrocyanosis-a-rare-manifestation-revealing-anti-pl12-syndrome
#1
Philippe Mertz, Mathilde Herber, Juliette Jeannel, Anne-Sophie Korganow, Aurélien Guffroy
A 69-year-old woman with asthenia, anorexia and weight loss lasting for several weeks was referred for a subacute onset of painful permanent acrocyanosis of the four extremities without Raynaud's phenomenon. She also had concomitantly developed grade I/IV (NYHA) dyspnea. A physical examination revealed livedoid acrocyanosis of distal phalanges of the hands (Fig. 1A), which was associated with flame-shaped hemorrhages (Fig. 1B, arrow-head), dilated capillaries at the proximal nailfolds (Fig. 1B, arrow), Gottron's sign over the MCP and PIP joints and a discrete heliotrope rash with a palpable V-shaped rash of the upper chest and forehead...
May 6, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29707197/case-report-acute-amyopathic-dermatomyositis-presenting-with-isolated-facial-edema
#2
Efthymia Pappa, Marina Gkeka, Asimina Protogerou, Leonidas Marinos, Chariclia Loupa, Constantinos Christopoulos
A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation...
2018: F1000Research
https://www.readbyqxmd.com/read/29571300/erythroderma-and-extensive-poikiloderma-a-rare-initial-presentation-of-dermatomyositis-a-case-report
#3
H M M T B Herath, B S D P Keragala, S P Pahalagamage, G H C C Janappriya, Aruna Kulatunga, C N Gunasekera
BACKGROUND: Dermatomyositis is a humoral-mediated inflammatory myopathy with symmetrical proximal muscle weakness and dermatological manifestations such as Gottron's papules, heliotrope rash, periungual abnormalities, and flagellate erythema. Erythroderma is a severe and potentially life-threatening dermatological condition with diffuse erythema and scaling involving more than 90% of the skin surface area. Poikiloderma vasculare atrophicans refers to mottled hyperpigmentation and hypopigmentation of the skin with in-between telangiectases and areas of atrophy and is considered a variant of mycosis fungoides...
March 24, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29526968/a-case-of-advanced-lung-adenocarcinoma-with-nvolumab-associated-dermatomyositis
#4
Fumiaki Kudo, Yasutaka Watanabe, Yuki Iwai, Chihiro Miwa, Yoshiaki Nagai, Hiromitsu Ota, Hiroki Yabe, Toshio Demitsu, Koichi Hagiwara, Nobuyuki Koyama, Shinichiro Koyama
We herein report a 42-year-old man with advanced lung adenocarcinoma and nivolumab-associated dermatomyositis. Nivolumab, an anticancer drug that is classified as an immune checkpoint inhibitor, often induces immune-related adverse events (irAEs). However, there have so far been no reports regarding nivolumab-associated dermatomyositis. This patient was diagnosed with dermatomyositis due to the presence of proximal muscle weakness with abnormal electromyography and magnetic resonance imaging findings; skin lesions, such as heliotrope rash, shawl sign, and periungual erythema; and an elevated serum aldolase level after nivolumab administration...
March 9, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29441299/livedo-racemosa-reticulated-ulcerations-panniculitis-and-violaceous-plaques-in-a-46-year-old-woman
#5
Alfredo Agulló, Brian Hinds, Mónica Larrea, Ignacio Yanguas
Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM - anti-melanoma differentiation-associated gene 5 (anti-MDA5). Patients with this serologic marker have a characteristic mucocutaneous phenotype consisting of skin ulceration among other signs. We describe the case of a 46-year-old woman with CADM, elevated anti-MDA5 autoantibodies, and unusual clinical features (livedo racemosa, florid acral edema) among the classical phenotype of MDA5 DM (arthralgias, ulcerations, panniculitis) and classical DM lesions (Gottron papules, heliotrope rash)...
January 2018: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/29412081/artificial-heliotropism-and-nyctinasty-based-on-optomechanical-feedback-and-no-electronics
#6
Bilge Baytekin, S Doruk Cezan, H Tarık Baytekin, Bartosz A Grzybowski
Although plants are typically not considered an inspiration for designing motile robots, they do perform a variety of intricate motion patterns, including diurnal cycles of sun tracking (heliotropism) and leaf opening (nyctinasty). In real plants, these motions are controlled by complex, feedback-based biological mechanisms that, to date, have been mimicked only in computer-controlled artificial systems. This work demonstrates both heliotropism and nyctinasty in a system in which few simple, but strategically positioned thermo-responsive springs and lenses form a feedback loop controlling these motions and substantiating a behavioral analogy to "plants...
February 2018: Soft Robotics
https://www.readbyqxmd.com/read/29330862/dermatomyositis-histopathologic-findings-of-parakeratosis-and-dermal-edema-revisited
#7
Lauren Levy, Heather Layher, Jennifer M McNiff, Christine J Ko
The cutaneous manifestations of dermatomyositis range from classical in the case of heliotrope rash and Gottron papules to less common papulosquamous and edematous/vesiculobullous lesions; histopathologic descriptions are dominated by interface dermatitis. We present a case of dermatomyositis with a combination of common and rare skin findings, both clinically and histologically. Increased awareness of papulosquamous and edematous lesions of dermatomyositis can help direct patient care. Although uncommon, confluent parakeratosis and dermal edema can be manifestations of dermatomyositis...
April 2018: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/29245346/two-independent-incidences-of-skin-metastases-in-the-umbilicus-and-abdominal-wall-in-ovarian-serous-adenocarcinoma-a-case-report-and-review-of-the-literature
#8
Hongyan Cheng, Chunmei Gao, Runtong Zhang, Zhaojie Yang, Guiyu Zhang
RATIONALE: Advanced ovarian cancer is usually associated with intra-abdominal metastases and while it commonly spreads directly to the omentum, intestine, liver, or other organs, it can also metastasize through the lymphatic channels and the hematogenous pathway. With an increasing number of invasive operations being performed with chemoradiotherapy, the incidence of extra-abdominal metastases has risen. Nevertheless, ovarian cancer with skin metastases is quite rare. PATIENT CONCERNS: We report a case of ovarian cancer with two independent incidences of skin metastases in the umbilicus and abdominal wall...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29199203/hemophagocytic-syndrome-complicated-with-dermatomyositis-controlled-successfully-with-infliximab-and-conventional-therapies
#9
Yoji Komiya, Tetsuya Saito, Fumitaka Mizoguchi, Hitoshi Kohsaka
A 57-year-old woman was admitted to our hospital because of a high fever, anemia, and hyperferritinemia. Since a bone marrow examination revealed hemophagocytosis, she was diagnosed with hemophagocytic syndrome (HPS). During treatment of HPS, a heliotrope rash and Gottron's sign appeared with elevated levels of serum aldolase. She also developed heart failure. She was diagnosed with dermatomyositis (DM) and associated myocarditis. Although the administration of glucocorticoids, calcineurin inhibitors, intravenous immunoglobulins, and etoposide ameliorated the clinical findings of DM and cytopenia, the fever and hyperferritinemia remained...
2017: Internal Medicine
https://www.readbyqxmd.com/read/29171576/an-elusive-case-of-dermatomyositis
#10
Tasnim Ahsan, Uzma Erum
Dermatomyositis is an inflammatory myopathy of unknown aetiology. Muscle involvement may eventuate later in the disease course in some patients, who may present with typical skin disease without clinical signs of myopathy and are referred to as dermatomyositis sine myositis. A 48 year old female presented with intermittent urticaria like rashes, diffuse asymmetrical swelling of proximal limbs, pain in small joints of hands and fatiguability. Initial laboratory work-up for immune markers was negative. Three years later, she developed heliotrope rash and periorbital oedema with no evidence of muscle weakness and was labeled as amyopathic dermatomyositis...
November 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/29027009/lipid-lowering-agent-triggered-dermatomyositis-and-polymyositis-a-case-series-and-literature-review
#11
REVIEW
Isabela Bruna Pires Borges, Marilda Guimarães Silva, Rafael Giovane Misse, Samuel Katsuyuki Shinjo
Lipid-lowering agent-triggered dermatomyositis (DM) or polymyositis (PM) is a rare event. Therefore, the aim of the present study was to describe a series of such cases. A retrospective cohort study of 5 DM and 4 PM cases triggered by prior exposure to lipid-lowering agents between 2001 and 2017 was carried out. All patients, except for two cases, had muscle biopsy compatible with inflammatory myopathy and no serum autoantibodies positive for anti-SRP or anti-HMGCoAR. Median age of the patients at time of diagnosis was 68 years...
February 2018: Rheumatology International
https://www.readbyqxmd.com/read/28912913/paraneoplastic-dermatomyositis-syndrome-presenting-as-dysphagia
#12
Emmanuel Ofori, Daryl Ramai, Mel Ona, Madhavi Reddy
Dermatomyositis (DM) is a rare autoimmune condition which predominantly affects females in the fifth and sixth decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with a heliotropic rash around the eyes, and Gottron's papules on extensor surfaces of the hands. While the pathophysiology of DM is still unclear, abnormal T- and B-cell immune activity has been reported. DM has been associated with malignancy and has been shown to appear before, concurrently with, or after diagnosis of cancer...
August 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28767186/diagnosis-of-subarachnoid-hemorrhage-time-for-a-paradigm-shift
#13
Nicole M Dubosh, Jonathan A Edlow
No abstract text is available yet for this article.
December 2017: Academic Emergency Medicine: Official Journal of the Society for Academic Emergency Medicine
https://www.readbyqxmd.com/read/28386737/the-epidemiology-of-dermatomyositis-in-northwestern-thrace-region-in-turkey-epidemiology-of-dermatomyositis-in-turkey
#14
Mehmet Ali Balci, Salim Donmez, Fatih Saritas, Volkan Bas, Omer Nuri Pamuk
Dermatomyositis (DM) is a rare disease that may affect the skeletal muscles and the skin. Literature data on its incidence and prevalence are limited. There are no data on its incidence or prevalence in Turkey. Patients diagnosed with DM at the Trakya University Medical Faculty, Department of Rheumatology from November 2004 to November 2014 were reviewed retrospectively. Patients' clinical and demographic features, laboratory data, treatment modalities, follow-up durations, disease courses, outcomes, and complications were evaluated...
September 2017: Rheumatology International
https://www.readbyqxmd.com/read/28251541/profile-of-pediatric-idiopathic-inflammatory-myopathies-from-a-tertiary-care-center-of-eastern-india
#15
Sumantra Sarkar, Tanushree Mondal, Arpan Saha, Rakesh Mondal, Supratim Datta
OBJECTIVES: To report data on Idiopathic inflammatory myopathies (IIM) from eastern India. METHODS: All IIM patients diagnosed over the last 5 y (2011-2016) were included through a retrospective review of records from the hospital and specialty clinic at Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata. RESULTS: Out of the 11 IIM patients, 9 had Juvenile dermatomyositis (JDM) and 2 had overlap myositis (OM) [with systemic lupus erythematosus (SLE) and scleroderma]...
April 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28210382/paraneoplastic-dermatomyositis-with-cutaneous-and-myopathic-disease-responsive-to-adrenocorticotropic-hormone-therapy
#16
Marisa Wolff, Christopher Mancuso, Karan Lal, Damian Dicostanzo, Charles Gropper
Dermatomyositis is a myopathic or amyopathic autoimmune connective tissue disease that presents with classic dermatologic findings ranging from: poikilodermatous photosensitivity (shawl sign), eyelid edema and violaceous-pigmentation (heliotrope sign), lichenoid eruptions on the knuckles and elbows (Gottron's sign), periungual telangiectasias, and ragged cuticles (Samitz sign). Up to 30 percent of adult-onset cases of dermatomyositis may represent a paraneoplastic syndrome warranting a thorough work-up for malignancy...
January 2017: Journal of Clinical and Aesthetic Dermatology
https://www.readbyqxmd.com/read/28190982/dermatomyositis-leading-to-necrotizing-vasculitis-a-perfect-response-to-applied-therapy
#17
Mahmood Akbaryan, Farideh Darabi, Zahra Soltani
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...
December 2016: International Journal of Biomedical Science: IJBS
https://www.readbyqxmd.com/read/28133992/the-family-impact-of-caring-for-a-child-with-juvenile-dermatomyositis
#18
Samantha Kountz-Edwards, Christa Aoki, Caitlin Gannon, Rowena Gomez, Matthew Cordova, Wendy Packman
Background Juvenile dermatomyositis (JDM), a rare autoimmune disease, accounts for more than 80% of idiopathic inflammatory myopathy childhood cases, making it the most common idiopathic inflammatory myopathy among children. The average age of onset is approximately 7 years and commonly leads a chronic course. Symptoms of JDM include cutaneous features (Gottron's rash, heliotrope rash, or nail fold capillary changes), musculoskeletal features, calcinosis and lipodystrophy (a symmetrical deficit of subcutaneous fatty tissue), and acanthosis (thickening of the skin)...
January 1, 2017: Chronic Illness
https://www.readbyqxmd.com/read/28058540/juvenile-dermatomyositis-a-tertiary-center-experience
#19
Kenan Barut, Pinar Ozge Avar Aydin, Amra Adrovic, Sezgin Sahin, Ozgur Kasapcopur
Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. 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...
February 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/27837048/cutaneous-improvement-in-refractory-adult-and-juvenile-dermatomyositis-after-treatment-with-rituximab
#20
RANDOMIZED CONTROLLED TRIAL
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
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