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Livedo reticularis

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https://www.readbyqxmd.com/read/29301476/the-cutaneous-manifestations-are-significantly-related-to-cerebrovascular-in-a-serbian-cohort-of-patients-with-hughes-syndrome
#1
L Stojanovich, A Djokovic, N Stanisavljevic, M Zdravkovic
Objectives To investigate a possible relationship between cerebrovascular, such as stroke and transient ischaemic attack, and various cutaneous manifestations (livedo reticularis, skin ulcerations, pseudovasculitis lesions, superficial cutaneous necrosis and digital gangrene) in antiphospholipid syndrome (APS). This report is based on a Serbian cohort of APS patients. Methods A total of 508 antiphospholipid syndrome APS patients were assessed: 360 with primary (PAPS) and 148 with APS associated with SLE (SAPS)...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29109739/a-case-of-polyarteritis-nodosa-presenting-as-rapidly-progressing-intermittent-claudication-of-right-leg
#2
Chathuranga Lakmal Fonseka, Sampath Rukshani Galappaththi, Dayakshi Abeyaratne, Nirmali Tissera, Lalith Wijayaratne
Background: Polyarteritis nodosa (PAN) is a medium vessel vasculitis which causes significant morbidity and mortality. Usually, it presents with constitutional symptoms with angiographic evidence of aneurysms or segmental stenosis of arteries of mesenteric or renal vasculature. It is exceedingly uncommon for PAN to present with symptomatic progressive intermittent claudication. Case Presentation: We describe a 60-year-old male who presented with rapidly progressive intermittent claudication of his right leg...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/29050535/antiphospholipid-antibodies-and-non-thrombotic-manifestations-of-systemic-lupus-erythematosus
#3
U İlgen, M E Yayla, A Ateş, İ E Okatan, E U Yurteri, M Torgutalp, A B D Keleşoğlu, T M Turgay, G Kınıklı
Objectives The aim of this study was to investigate the association between antiphospholipid antibodies and non-thrombotic and non-gestational manifestations of systemic lupus erythematosus. Methods Systemic lupus erythematosus patients with persistently positive antiphospholipid antibodies or lupus anticoagulant were identified and grouped as systemic lupus erythematosus with antiphospholipid syndrome (SLE-APS), systemic lupus erythematosus with positive antiphospholipid antibodies/lupus anticoagulant without antiphospholipid syndrome (SLE-aPL), and systemic lupus erythematosus with negative aPLs (SLE-No aPL)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28974505/hematopoietic-stem-cell-transplantation-rescues-the-hematological-immunological-and-vascular-phenotype-in-dada2
#4
MULTICENTER STUDY
Hasan Hashem, Ashish R Kumar, Ingo Müller, Florian Babor, Robbert Bredius, Jignesh Dalal, Amy P Hsu, Steven M Holland, Dennis D Hickstein, Stephen Jolles, Robert Krance, Ghadir Sasa, Mervi Taskinen, Minna Koskenvuo, Janna Saarela, Joris van Montfrans, Keith Wilson, Barbara Bosch, Leen Moens, Michael Hershfield, Isabelle Meyts
Deficiency of adenosine deaminase 2 (DADA2) is caused by biallelic deleterious mutations in CECR1 DADA2 results in variable autoinflammation and vasculopathy (recurrent fevers, livedo reticularis, polyarteritis nodosa, lacunar ischemic strokes, and intracranial hemorrhages), immunodeficiency and bone marrow failure. Tumor necrosis factor-α blockade is the treatment of choice for the autoinflammation and vascular manifestations. Hematopoietic stem cell transplantation (HSCT) represents a potential definitive treatment...
December 14, 2017: Blood
https://www.readbyqxmd.com/read/28961286/asymptomatic-cutaneous-polyarteritis-nodosa-treatment-options-and-therapeutic-guidelines
#5
Kate Ross, Jessika Contreras, David Aung-Din, Mary Lien
Cutaneous polyarteritis nodosa (CPAN) is a rare cutaneous small- to medium-vessel vasculitis of unknown etiology. Clinically it ranges in manifestation from livedo reticularis to large cutaneous ulcers and necrosis. Prognosis is favorable and progression to systemic polyarteritis nodosa is rare. There are multiple treatment options, none of which have proven to be definitively effective. Cutaneous polyarteritis nodosa has been associated with abnormal antibody testing with elevations of antiphospholipid cofactor antibody, lupus anticoagulant, anticardiolipin antibody, and anti-β2-glycoprotein I-dependent cardiolipin antibodies, as well as elevated anti-phosphatidylserine-prothrombin complex antibody...
August 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28882931/livedoid-eruption-in-a-patient-affected-by-t-%C3%AE-%C3%AE-large-granular-lymphocyte-leukaemia
#6
Giulia Biolo, Giorgia Sacco, Irene Russo, Mauro Alaibac
Livedo is an ischaemic dermopathy characterised by a reddish-blue to violaceous mottling of the skin with a net-like reticular appearance. Livedo has been described in association with several medical conditions including lymphoproliferative disorders. Here, we describe the case of a 60-year-old woman who was presented with asymptomatic and persistent livedoid eruption on her trunk, lower and upper extremities as manifestation of an indolent form of T-γδ large granular lymphocyte leukaemia. To the best of our knowledge, this is the first report describing the association between livedo reticularis and T-γδ large granular lymphocyte leukaemia...
September 7, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28871434/cutaneous-and-mucosal-manifestations-of-sj%C3%A3-gren-s-syndrome
#7
REVIEW
Elena Generali, Antonio Costanzo, Carlo Mainetti, Carlo Selmi
Sjögren's syndrome is currently considered an "autoimmune epithelitis," as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren's syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases...
September 4, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28859746/oxalosis-in-a-patient-with-livedo-reticularis
#8
Meriam Triki, Meriem Ksentini, Rim Kallel, Emna Bahloul, Faiçal Jarraya, Abderrahmen Masmoudi, Tahya Boudawara
A 27-year-old man with terminal renal failure requiring peritoneal dialysis for the past 2 years was referred to the dermatologist for evaluation of red violaceous macular skin lesions consistent with livedo reticularis. These lesions had appeared suddenly on his legs (Figure 1). He had first experienced recurrent nephrolithiasis at the age of 14. Results from urine analysis and abdominal ultrasound revealed chronic kidney failure. Because the patient had a sister with similar findings, primary hyperoxaluria (PH) was suspected and genetic testing was performed in all members of his family...
2017: Skinmed
https://www.readbyqxmd.com/read/28825580/-calciphylaxis
#9
Nicolás Raimondi, Jorge J Del Vecchio, Mauricio Ghioldi, Emanuel Uzair
Calciphylaxis is vasculopathy characterized by ischemia and painful skin necrosis due to calcification and intimal fibroplasia of thrombosis of the panicular arterioles. It most frequently compromises patients with terminal chronic renal failure and has a high mortality rate. Biopsy of skin lesions is used as a diagnostic method. No specific laboratory findings have been recorded. Skin lesions usually begin in the extremities like a painful purplish mottling similar to "livedo reticularis". The natural evolution is to ulcers and bedsores...
2017: Medicina
https://www.readbyqxmd.com/read/28770705/are-the-cutaneous-manifestations-in-patients-with-primary-antiphospholipid-syndrome-a-marker-for-predicting-lung-manifestations
#10
Milica Kontic, Ljudmila Stojanovich, Milena Mijailović-Ivković, Mladen Velinović, Jasminka Srnka, Marija Zdravkovic
OBJECTIVES: The aim of this study was to investigate association between pulmonary and skin manifestations in a large group of patients with primary antiphospholipid syndrome (PAPS) as well as their connection with antiphospholipid antibodies. METHODS: Our prospective study comprises of 390 patients with primary APS. Antiphospholipid antibody (aPL) analysis included detection of aCL (IgG/IgM), ß2GPI (IgG/IgM) and LA. Distinct pulmonary and skin associations were determined, as well as their associations with aPL...
July 17, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28769114/diagnosing-antiphospholipid-syndrome-extra-criteria-manifestations-and-technical-advances
#11
REVIEW
Savino Sciascia, Mary-Carmen Amigo, Dario Roccatello, Munther Khamashta
First described in the early 1980s, antiphospholipid syndrome (APS) is a unique form of acquired autoimmune thrombophilia in which patients present with clinical features of recurrent thrombosis and pregnancy morbidity and persistently test positive for the presence of antiphospholipid antibodies (aPL). At least one clinical (vascular thrombosis or pregnancy morbidity) and one lab-based (positive test result for lupus anticoagulant, anticardiolipin antibodies and/or anti-β2-glycoprotein 1 antibodies) criterion have to be met for a patient to be classified as having APS...
September 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#12
Uroosa Ibrahim, Shiksha Kedia, Gwenalyn Garcia, Jean Paul Atallah
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28593703/effect-of-ethnicity-on-clinical-presentation-and-risk-of-antiphospholipid-syndrome-in-roma-and-caucasian-patients-with-systemic-lupus-erythematosus-a-multicenter-cross-sectional-study
#13
Victoria Manzano-Gamero, Alfredo J Pardo-Cabello, José A Vargas-Hitos, Mónica Zamora-Pasadas, Nuria Navarrete-Navarrete, José M Sabio, Laura Jáimez-Gámiz, Raquel Ríos-Fernandez, Norberto Ortego-Centeno, M Mar Ayala-Gutierrez, Enrique de Ramón, Agustín Colodro-Ruíz, Luisa Micó-Giner, María J Castillo-Palma, Ángel Robles-Marhuenda, Juan de Dios Luna-Del Castillo, Juan Jiménez-Alonso
AIM: To determine if there are ethnic differences in the prevalence of antiphospholipid syndrome (APS), clinical presentation and autoantibody profile between Roma and Caucasian patients with systemic lupus erythematosus (SLE). METHOD: A cross-sectional study was conducted including data from Roma and Caucasian SLE patients consecutively attending six hospitals in Spain. Socio-demographic characteristics, prevalence of APS, clinical and analytical features of SLE and APS were compared between ethnic groups...
June 7, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28581281/a-case-of-rheumatoid-vasculitis-involving-hepatic-artery-in-early-rheumatoid-arthritis
#14
Ji Eun Lee, In Je Kim, Min Sun Cho, Jisoo Lee
Rheumatoid vasculitis is a rare, but most serious extra-articular complications of long-standing, seropositive rheumatoid arthritis (RA). Vasculitis of hepatic artery is an extremely rare but severe manifestation of rheumatoid vasculitis. A 72-year-old woman who presented with polyarthralgia for 2 months was diagnosed with early RA. Since she had manifestations of livedo reticularis, and liver dysfunction which was atypical for RA patients, a percutaneous needle liver biopsy was performed revealing arteritis of a medium-sized hepatic artery...
July 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28552872/mononeuritis-multiplex-in-a-patient-with-cutaneous-arteritis-diagnosed-by-skin-biopsy
#15
Kenji Isahaya, Makoto Shiraishi, Keita Tanaka, Rie Sasaki, Tamio Kawakami, Yasuhiro Hasegawa
A 55-year-old man was admitted with paralysis of the left lower leg. He had purpura in the left lower extremity for three years, left calf pain for two years, and dysesthesia in the left plantar region and first toe for one year. A physical examination revealed livedo reticularis on the left leg and mononeuritis multiplex was diagnosed in the bilateral tibial and left peroneal nerve area. Anti-neutrophil cytoplasmic antibody was negative. A nerve conduction study showed decreased amplitude of compound muscle-action potential in the bilateral tibial and the left peroneal nerve, sensory nerve action potential in the bilateral sural nerve...
June 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28536734/livedo-reticularis-on-the-lower-limbs-in-a-patient-with-lupus-erythematosus-a-quiz
#16
Elodie Miquelestorena-Standley, Alia Saleh, Anne Lataste, Christophe Monegier du Sorbier, Laurent Machet
is missing (Quiz).
October 2, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28522451/ada2-deficiency-dada2-as-an-unrecognised-cause-of-early-onset-polyarteritis-nodosa-and-stroke-a-multicentre-national-study
#17
MULTICENTER STUDY
Roberta Caorsi, Federica Penco, Alice Grossi, Antonella Insalaco, Alessia Omenetti, Maria Alessio, Giovanni Conti, Federico Marchetti, Paolo Picco, Alberto Tommasini, Silvana Martino, Clara Malattia, Romina Gallizi, Rosa Anna Podda, Annalisa Salis, Fernanda Falcini, Francesca Schena, Francesca Garbarino, Alessia Morreale, Manuela Pardeo, Claudia Ventrici, Chiara Passarelli, Qing Zhou, Mariasavina Severino, Carlo Gandolfo, Gianluca Damonte, Alberto Martini, Angelo Ravelli, Ivona Aksentijevich, Isabella Ceccherini, Marco Gattorno
OBJECTIVES: To analyse the prevalence of CECR1 mutations in patients diagnosed with early onset livedo reticularis and/or haemorrhagic/ischaemic strokes in the context of inflammation or polyarteritis nodosa (PAN). Forty-eight patients from 43 families were included in the study. METHODS: Direct sequencing of CECR1 was performed by Sanger analysis. Adenosine deaminase 2 (ADA2) enzymatic activity was analysed in monocyte isolated from patients and healthy controls incubated with adenosine and with or without an ADA1 inhibitor...
October 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28073520/livedo-reticularis-in-the-legs
#18
Ana Varela-Veiga, Carlos Aliste, Hugo A Vázquez-Veiga, María Dolores Sánchez-Aguilar
No abstract text is available yet for this article.
October 23, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28012321/recurrent-spontaneous-pneumoperitoneum-a-surgical-dilemma
#19
Polly Estridge, Jacob A Akoh
INTRODUCTION: Spontaneous pneumoperitoneum describes free air within the peritoneal cavity in the absence of iatrogenic causes or a perforated viscus. This report describes a rare case in which despite raised inflammatory markers, a trial of conservative management proved adequate. CASE REPORT: AM is a 36-year old woman who presented multiple times with abdominal pain and radiologically proven pneumoperitoneum. Her medical history included self catheterisation and cerebrovascular stenosis (Moyamoya disease), asymptomatic gallstones, livedo reticularis and peptic ulceration...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27930432/retinal-findings-in-a-case-of-presumed-cutis-marmorata-telangiectatica-congenita
#20
Eman A Taleb, Manish P Nagpal, Navneet S Mehrotra, Kalyani Bhatt
PURPOSES: To correlate the clinical picture and fundus fluorescein angiography findings in case of presumed cutis marmorata telangiectatica congenita. METHODS: A 41-year-old woman with presumed cutis marmorata telangiectatica congenita who is a known case of hypothyroidism for the last 6 years and has bilateral peripheral retinal vascular abnormalities, peripheral retinal nonperfusion on fluorescein angiography and bilateral optic disk drusen. RESULT: A patient presented with blurring of vision in both eyes for last 6 months...
December 7, 2016: Retinal Cases & Brief Reports
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