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Livedo reticularis

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https://read.qxmd.com/read/31669478/atheroembolism-to-the-breast
#1
Shruti Zaveri, Lucyna Z Price, Haley Tupper, Rami O Tadros
We report the case of a woman presenting with livedo reticularis of the breast who was found to have atheroembolism to the breast following upper extremity percutaneous access. Atheroembolism is the embolization of cholesterol crystals off an atherosclerotic plaque that can occur spontaneously or as a result of vascular intervention. This is a unique presentation of an otherwise well-described complication of vascular catheterization, and we propose that livedo reticularis of the breast can be interpreted as a sign of atheroembolism in the appropriate clinical context...
October 24, 2019: Annals of Vascular Surgery
https://read.qxmd.com/read/31580546/multifaceted-approach-to-a-rare-clinical-case-of-calciphylaxis-in-a-renal-transplant-recipient
#2
Carlo Massimetti, Antonio Bellasi, Antonio Modoni, Vito Gomes, Sandro Feriozzi
Calcific uremic arteriolopathy (CUA) is a highly morbid condition usually found in ESRD patients that has rarely been reported after renal transplantation and renal function restoration. Furthermore, little is known about the optimal management of CUA in this setting. Herein, we report on the clinical case of AB, a 70-year-old woman who developed CUA after renal transplantation and renal function restoration. However, other risk factors for CUA such as diabetes and warfarin treatment, due to mechanical aortic valve implantation, were present...
September 24, 2019: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://read.qxmd.com/read/31553839/livedo-reticularis-in-cold-agglutinin-disease
#3
Konika Sharma, Anush Patel
New England Journal of Medicine, Volume 381, Issue 13, September 2019.
September 26, 2019: New England Journal of Medicine
https://read.qxmd.com/read/31522599/a-homozygote-novel-l451w-mutation-in-cecr1-gene-causes-deficiency-of-adenosine-deaminase-2-in-a-pediatric-patient-representing-with-chronic-lymphoproliferation-and-cytopenia
#4
Rabia Miray Kisla Ekinci, Sibel Balcı, Atil Bisgin, Ilgen Sasmaz, Goksel Leblebisatan, Faruk Incecik, Mustafa Yilmaz
Deficiency of Adenosine Deaminase 2 (DADA2) is a monogenic autoinflammatory disorder characterized by livedo reticularis, skin ulcers, subcutaneous rash, aphthous ulcers, and leukocytoclastic vasculitis, neurological signs such as early onset stroke and polyneuropathy. A minority of DADA2 patients suffer from severe cytopenia and lymphoproliferation. Herein, we report an adolescent patient, followed up as having a hematological disorder for many years, eventually diagnosed as having DADA2. In view of the presence of elevated acute phase reactants, hepatosplenomegaly, low IgM level, lymphopenia, anemia, and neutropenia, and a subtle neurological involvement we considered DADA2 diagnosis...
September 14, 2019: Pediatric Hematology and Oncology
https://read.qxmd.com/read/31517743/paracentral-acute-middle-maculopathy-after-aortic-aneurysm-repair
#5
Leonardo Lando, David Leonardo C Isaac, Marcos P Avila
PURPOSE: We report a case of paracentral acute middle maculopathy secondary to cholesterol embolization syndrome after an endovascular aortic aneurism repair (EVAR). METHODS: Functional and anatomical damage was assessed by Snellen visual acuity, fundoscopy, and optical coherence tomography. RESULTS: A 67-year-old man presented with sudden vision loss in the right eye for one day with concomitant livedo reticularis in both feet. His medical history included an abdominal EVAR repair three days before the symptoms...
September 10, 2019: Retinal Cases & Brief Reports
https://read.qxmd.com/read/31477950/a-simplified-understanding-of-the-black-swan-anti-phospholipid-antibody-syndrome
#6
Binit Vaidya, Shweta Nakarmi, Rakshya Joshi, Rikesh Baral
Anti-phospholipid Antibody Syndrome or Hugh's syndrome is a heterogeneous disorder, first fully described in 1980s. The syndrome is caused by the presence of specific antibodies against phospholipid binding plasma proteins in the serum of the patient, with or without underlying autoimmune diseases, that causes prolongation of tests of coagulation. High index of clinical suspicion is required for diagnosis of Anti-phospholipid Antibody Syndrome. Stroke or myocardial infarction in young, unprovoked recurrent deep vein thrombosis and recurrent pregnancy loss are typical scenarios where Anti-Phospholipid Antibody Syndrome should be suspected...
March 2019: JNMA; Journal of the Nepal Medical Association
https://read.qxmd.com/read/31377769/immunosuppressive-therapy-to-reduce-mitral-regurgitation-in-libman-sacks-endocarditis-a-case-report
#7
Kenichi Ishizu, Akihiro Isotani, Kyohei Yamaji, Kenji Ando
BACKGROUND: Libman-Sacks endocarditis is a cardiac manifestation of systemic lupus erythematosus (SLE) and is characterized by non-bacterial verrucous vegetations, causing valvular stenosis and/or regurgitation. The effectiveness of immunosuppressive therapy for valve dysfunction due to Libman-Sacks endocarditis has not been reported. CASE SUMMARY: A 67-year-old woman with a history of chronic atrial fibrillation was emergently admitted with acute decompensated heart failure...
August 4, 2019: European Heart Journal. Case Reports
https://read.qxmd.com/read/31145079/relax-from-the-holiday-on-the-edge-of-the-lagoon-turquoise-waters-and-red-legs
#8
A Haar, A C Garcia, J Morin, J J Morand, J E Blatteau
We report a case of livedo reticularis of the lower limbs in a 30-year-old woman scuba diver after a diving session. Consideration of the differential diagnoses, the clinical picture, and the course of the symptoms led to a diagnosis of cutaneous decompression sickness. This accident, which resolved favorably in this scuba diver without any right-to-left shunt, is an alarm signal that calls for her to comply strictly with safety rules before resuming scuba diving, to avoid a more serious accident.
May 29, 2019: Médecine et Santé Tropicales
https://read.qxmd.com/read/31120013/-sneddon-s-syndrome-in-a-young-woman-with-antiphospholipid-syndrome-ischaemic-apoplexy-and-epileptic-seizures
#9
Christian Stenør, Morten Togo Sørensen
In this case report, a 28-year-old woman known with slight aortic regurgitation presented with partial complex epileptic seizures. On examination, livedo reticularis was noted, and cerebral MRI scans showed signs of clinical silent old lacunar infarctions. She was persistently triple positive for antiphospholipid antibodies in high titres and fulfilled the antiphospholipid syndrome criteria. The patient was diagnosed with Sneddon's syndrome, which is a rare thrombotic vasculopathy characterised by the combination of cerebrovascular disease with livedo reticularis...
May 6, 2019: Ugeskrift for Laeger
https://read.qxmd.com/read/30745637/generalized-livedo-reticularis-a-rare-variety
#10
Pallavi Goyal, Surabhi Dayal, Priyadarshini Sahu
Livedo reticularis can present with progressive ischemia and diffuse cutaneous involvement with or without any evidence of systemic diseases. Livedo reticularis (LR) is a livedoid discoloration of the skin in a reticular pattern. We report the case of a 30-year-old male who presented with an asymptomatic, red-colored, net-like rash all over the body for 4 years. Laboratory investigations were performed to rule out any systemic involvement. Biopsy showed perivascular mononuclear cell infiltrate and occasional arteriole showed thickening of the wall with obliteration of the lumen and extensive collagenization in dermis, suggesting a diagnosis of LR...
January 2019: Indian Journal of Dermatology
https://read.qxmd.com/read/30739544/peripheral-nervous-system-involvement-in-systemic-lupus-erythematosus-a-retrospective-study-on-prevalence-associated-factors-and-outcome
#11
A Bortoluzzi, M Piga, E Silvagni, E Chessa, A Mathieu, M Govoni
BACKGROUND: Despite its potentially significant impact on disease outcome, peripheral nervous system involvement in systemic lupus erythematosus has received little attention. OBJECTIVE: The objective of this study was to assess the prevalence and clinical features of peripheral nervous system involvement in a large cohort of systemic lupus erythematosus patients. METHODS: The records of systemic lupus erythematosus patients examined at two tertiary referral centres over a period of 14 years (from 2000 to 2014) were analyzed...
April 2019: Lupus
https://read.qxmd.com/read/30645994/adenosine-deaminase-two-and-immunoglobulin-m-accurately-differentiate-adult-sneddon-s-syndrome-of-unknown-cause
#12
Gustavo C Santo, Inês Baldeiras, Rita Guerreiro, Joana A Ribeiro, Rosário Cunha, Taryn Youngstein, Sira Nanthapisal, João Leitão, Carolina Fernandes, Francisco Caramelo, Maria do Rosário Almeida, José Brás, Isabel Santana
BACKGROUND: The association that exists between livedo reticularis (LR) and stroke is known as Sneddon's syndrome (SnS). The disorder is classified as primary SnS (PSnS), if the cause remains unknown and secondary SnS. The condition is rare and it occurs mainly sporadically. In 2014, 2 independent teams described a new genetic disorder with childhood-onset, which was called deficiency of adenosine deaminase 2 (DADA2), characterized by recurrent fevers and vascular pathologic features that included LR and stroke...
January 15, 2019: Cerebrovascular Diseases
https://read.qxmd.com/read/30524428/presence-of-immune-complexes-of-igg-igm-bound-to-b2-glycoprotein-i-is-associated-with-non-criteria-clinical-manifestations-in-patients-with-antiphospholipid-syndrome
#13
Dolores Pérez, Ljudmila Stojanovich, Laura Naranjo, Natasa Stanisavljevic, Gordana Bogdanovic, Manuel Serrano, Antonio Serrano
Background: Antiphospholipid syndrome (APS) is an acquired autoimmune disorder defined by the presence of both clinical (thromboembolic events or pregnancy morbidity) and laboratory (antiphospholipid antibodies, aPL) manifestations. Despite their importance, several clinical manifestations strongly associated with APS such as livedo reticularis (LR), thrombocytopenia, sicca-ophthalmic(sicca), heart, or neurological manifestations are not included in the APS clinical classification criteria. Circulating immune complexes (CIC) formed by Beta-2-glycoprotein I (B2GPI) and aPL (B2-CIC) have been described and their presence has been related with thrombotic events...
2018: Frontiers in Immunology
https://read.qxmd.com/read/30375212/antiphospholipid-syndrome-and-pregnancy
#14
Rosanna Satta, Gabriele Biondi
Antiphospholipid syndrome (APS) is an autoimmune thrombophilic condition characterized by the onset of venous and/or arterial thrombosis, often multiple, and pregnancy morbidity in a background of antiphospholipid antibodies (aPL) positivity. Some patients can be carrier of aPL with no clinical symptoms, in other cases clinical manifestation can range from the classical presentation to an acute life-threatening condition named Catastrophic APS. APS can be considered as primary or associated to other disease, however pregnancy acts as a triggering factor on a susceptible background that lead to the clinical manifestations through immunological and non-immunological mechanism...
October 29, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://read.qxmd.com/read/30372725/amantadine-induced-livedo-reticularis-in-a-child-treated-off-label-for-neurobehavioral-disorders
#15
Kenneth J Helmandollar, Kara R Hoverson, Jon H Meyerle
We report the case of an 8-year-old boy who was taking amantadine off label for multiple childhood neurobehavioral disorders and subsequently developed livedo reticularis. Although this side effect is well-described in adult patients taking amantadine for Parkinson disease, it is now being seen in children as the off-label use of amantadine is expanded to this population.
September 2018: Cutis; Cutaneous Medicine for the Practitioner
https://read.qxmd.com/read/30282668/management-of-antiphospholipid-syndrome
#16
Imad Uthman, Mohammad Hassan A Noureldine, Guillermo Ruiz-Irastorza, Munther Khamashta
Antiphospholipid syndrome, also known as 'Hughes Syndrome', is an autoimmune disease characterised by a set of clinical manifestations, almost all of which are direct or indirect sequelae of a hypercoagulable state involving the venous, and to a lesser extent the arterial vasculature. The incidence and prevalence of antiphospholipid syndrome are estimated at approximately 5 de novo cases per 100 000 per year and 40-50 cases per 100 000 individuals, respectively. The clinical spectrum of antiphospholipid syndrome involves haematological (thrombocytopaenia, venous thrombosis), obstetrical (recurrent pregnancy loss), neurological (stroke, transient ischaemic attack, migraine, seizures, cognitive dysfunction, chorea, transverse myelitis, multiple sclerosis), cardiovascular (cardiac valve disease), dermatological (livedo reticularis and racemosa, skin ulceration and necrosis), renal (glomerulonephritis, renal thrombotic microangiopathy) and orthopaedic (avascular necrosis of bones, non-traumatic fractures) manifestations, among others...
February 2019: Annals of the Rheumatic Diseases
https://read.qxmd.com/read/30160190/a-microvascular-myocardial-infarction-in-a-16-year-old-girl-with-antiphospholipid-syndrome-a-case-report
#17
Jean-Philippe Riga, Attilio Leone, Frédéric Lambot, Olivier Cappeliez, Alain Friart
OBJECTIVE AND IMPORTANCE: The antiphospholipid syndrome can manifest itself by silent (or not) myocardial infarction. CLINICAL PRESENTATION: We report the case of a 16-year-old girl who presented a myocardial infarction for whom a coronary-computer tomography did not reveal any coronary abnormalities or obstruction. She had a livedo reticularis on her physical exam. INTERVENTION: The echocardiography showed a normal left ventricular function and a mild eccentric mitral regurgitation...
August 30, 2018: Acta Clinica Belgica
https://read.qxmd.com/read/30038614/a-mutation-outside-the-dimerization-domain-causing-atypical-sting-associated-vasculopathy-with-onset-in-infancy
#18
Rohit G Saldanha, Katherine R Balka, Sophia Davidson, Brynn K Wainstein, Melanie Wong, Rebecca Macintosh, Christine K C Loo, Martin A Weber, Vasanth Kamath, Fiona Moghaddas, Dominic De Nardo, Paul Edgar Gray, Seth Lucian Masters
Background: Mutations in the gene encoding stimulator of interferon genes (STING) underlie a type I interferon (IFN) associated disease, STING-associated vasculopathy with onset in infancy (SAVI). Patients suffer cutaneous vasculopathy and interstitial lung disease, but are not known to suffer life-threatening infection. Case: We describe a child who presented with Pneumocystis jirovecii pneumonia in early life, from which he recovered. He went on to suffer failure to thrive, developmental delay, livedo reticularis, and vesicular rash, but without cutaneous vasculitis, and with normal C-reactive protein and erythrocyte sedimentation rates...
2018: Frontiers in Immunology
https://read.qxmd.com/read/29988314/cognitive-and-psychiatric-changes-as-first-clinical-presentation-in-sneddon-syndrome
#19
Giorgio Fabiani, Raul Martins, Gelson Luis Koppe, Zeferino Demartini, Luana Antunes Maranha Gatto
Sneddon syndrome (SS) is a rare progressive non-inflammatory thrombotic vasculopathy affecting small/medium-sized blood vessels of unknown origin. It is strongly associated with the presence of antiphospholipid antibodies (AA). The presence of livedo reticularis and cerebrovascular disease are hallmark features. The condition is far more common in young women. We report a case of SS in a 43 year-old male with a two-year history of progressive cognitive impairment consistent with dementia syndrome, and major personality changes, besides livedo reticularis and cerebral angiographic pattern of vasculitis...
April 2018: Dementia & Neuropsychologia
https://read.qxmd.com/read/29951947/deficiency-of-adenosine-deaminase-2-dada2-updates-on-the-phenotype-genetics-pathogenesis-and-treatment
#20
REVIEW
Isabelle Meyts, Ivona Aksentijevich
Deficiency of ADA2 (DADA2) is the first molecularly described monogenic vasculitis syndrome. DADA2 is caused by biallelic hypomorphic mutations in the ADA2 gene that encodes the adenosine deaminase 2 (ADA2) protein. Over 60 disease-associated mutations have been identified in all domains of ADA2 affecting the catalytic activity, protein dimerization, and secretion. Vasculopathy ranging from livedo reticularis to polyarteritis nodosa (PAN) and life-threatening ischemic and/or hemorrhagic stroke dominate the clinical features of DADA2...
July 2018: Journal of Clinical Immunology
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