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Hypocomplementemic urticarial vasculitis

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https://www.readbyqxmd.com/read/29594143/a-case-of-crescentic-glomerulonephritis-complicated-with-hypocomplementemic-urticarial-vasculitis-syndrome-and-anca-associated-vasculitis
#1
Marenao Tanaka, Norihito Moniwa, Tomohiro Mita, Toshiyuki Tobisawa, Tamaki Matsumoto, Atsushi Mochizuki, Tomohisa Yamashita, Toshiyuki Yano, Masato Furuhashi, Tetsuji Miura
Systemic urticaria in a 64-year-old woman was diagnosed as leukocytoclastic vasculitis by a punch biopsy of the skin. Her physical findings improved after prescription of prednisolone at a dose of 20 mg/day, but the skin rash relapsed with renal dysfunction, proteinuria, and hematuria when the dose of prednisolone was reduced over a period of 9 months to 1 mg/day. She was admitted to our institute for further examination, when urinary protein and plasma creatinine levels were 0.8 g/day and 1.7 mg/dL, respectively...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29406049/hypocomplementemic-urticarial-vasculitis-syndrome-with-crescentic-glomerulonephritis
#2
Sohail Abdul Salim, Tauqeer Yousuf, Asha Patel, Tibor Fülöp, Mohit Agarwal
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation...
February 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29329127/cutis-laxa-acquisita-after-urticarial-vasculitis-in-sle-patients
#3
Kimberly B Golisch, Silvija P Gottesman, Patricia Ferrer, Keliegh S Culpepper
Cutis laxa is a rare connective tissue disease involving damage to dermal elastic fibers creating a clinical appearance of loose, sagging skin. The condition can be either acquired or genetic. Autoimmune diseases, neoplasms, infections, and medications have been proposed as the cause of, or in association with, the acquired form. In nearly 50% of cases, erythematous plaques present before the onset of cutis laxa. Separately, urticarial vasculitis and systemic lupus erythematosus have been linked to cutis laxa acquisita...
January 11, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29199964/a-case-of-hypocomplementemic-urticarial-vasculitis-syndrome-successfully-treated-with-omalizumab
#4
E Nucera, F Basta, A Buonomo, S Mezzacappa, D P Margiotta, R Antonelli Incalzi, D Schiavino
No abstract text is available yet for this article.
2017: Journal of Investigational Allergology & Clinical Immunology
https://www.readbyqxmd.com/read/29115092/hypocomplementemic-urticarial-vasculitis-syndrome-with-membranous-nephropathy-case-report
#5
Su Woong Jung, Yun Young Choi, In Seung Choi, Seulki Kim, Kyung Hwan Jeong, Ran Song, Sang Hoon Lee, Hyung In Yang, Seung Jae Hong, Yeon Ah Lee
Urticarial vasculitis is a rare disorder that principally manifests with recurrent urticarial, sometimes hemorrhagic, skin lesions and/or angioedema. Its clinical presentation is not always limited to cutaneous lesions and it can potentially affect other organs, such as the joints, lungs, kidneys, and eyes. Systemic involvement can either be present at the onset of disease or develop over time. In cases with systemic manifestations, urticarial vasculitis is more likely to be associated with a low complement level...
December 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29114186/clinical-utility-of-anti-c1q-antibody-in-primary-and-secondary-vasculitic-conditions
#6
Kabeerdoss Jayakanthan, And Nikhil Gupta, John Mathew, Raheesh Ravindran, Gowri Mahasampth, Debashish Danda
Objective: Anti-C1q antibodies (Anti-C1q Ab) are seen in hypocomplementemic urticarial vasculitis syndrome (HUVS), infection-associated vasculitis such as hepatitis C virus-related vasculitis and in autoimmune diseases such as rheumatoid vasculitis, polyarteritis nodosa, giant cell arteritis, vascular Behcet's disease, and cryoglobulin associated vasculitis. Aim of this study is to evaluate the presence of Anti-C1q Ab in vasculitis and to determine if any difference exists between primary and secondary vasculitis in relation to this antibody...
November 2017: International Journal of Health Sciences
https://www.readbyqxmd.com/read/29092674/autoantibodies-against-complement-components-in-systemic-lupus-erythematosus-role-in-the-pathogenesis-and-clinical-manifestations
#7
M H Hristova, V S Stoyanova
Many complement structures and a number of additional factors, i.e. autoantibodies, receptors, hormones and cytokines, are implicated in the complex pathogenesis of systemic lupus erythematosus. Genetic defects in the complement as well as functional deficiency due to antibodies against its components lead to different pathological conditions, usually clinically presented. Among them hypocomplementemic urticarial vasculitis, different types of glomerulonephritis as dense deposit disease, IgA nephropathy, atypical haemolytic uremic syndrome and lupus nephritis are very common...
December 2017: Lupus
https://www.readbyqxmd.com/read/28776364/jaccoud-s-arthropathy-an-unusual-manifestation-of-idiopathic-retroperitoneal-fibrosis-rapid-improvement-of-symptoms-after-tocilizumab-treatment
#8
M Benucci, A Damiani, F Li Gobbi, V Grossi, M Infantino, A Arena, M Manfredi
Jaccoud's arthropathy (JA) is a chronic, non erosive, rheumatoid-like deformity associated with rheumatic fever (RF) and systemic lupus erythematosus and with other diseases such as psoriatic arthritis, connective tissue diseases, hypocomplementemic urticarial vasculitis, infections, sarcoidosis and neoplasia. We described a case of JA in a patient with cutaneous psoriasis but with a particular disease evolution associated with idiopathic retropritoneal fibrosis (IRF), evaluated with computed tomography, magnetic resonance and 18F-FDG PET/ CT...
August 3, 2017: Reumatismo
https://www.readbyqxmd.com/read/28457680/-hypocomplementemic-urticarial-vasculitis
#9
M Jachiet, B Flageul, J-D Bouaziz, M Bagot, B Terrier
Hypocomplementemic urticarial vasculitis (HUV), called anti-C1q vasculitis in the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides, is a rare systemic vasculitis of unknown etiology, affecting small vessels. HUV is characterized by urticarial lesions, hypocomplementemia and systemic manifestations, mostly musculoskeletal and ocular, but also gastrointestinal, pulmonary and kidney involvement. Anti-C1q antibodies are detected in only half of the patients, and low C1q seems to represent a more sensitive marker...
February 2018: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28273376/omalizumab-does-not-improve-skin-lesions-in-a-patient-with-hypocomplementemic-urticarial-vasculitis-syndrome
#10
LETTER
S Aurich, J C Simon, R Treudler
No abstract text is available yet for this article.
March 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27852431/urticarial-vasculitis-in-the-childhood-with-c2-hypocomplementenemia-a-rare-case
#11
Y Keyla Chan, R F J Criado, P R Criado, C D A S Machado, C Speyer
We report a first case of hypocomplementemic urticarial vasculitis of C2 fraction in a child, with cutaneous manifestation only, with no reports in scientific literature.
November 2016: European Annals of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27821515/an-autosomal-recessive-dnase1l3-related-autoimmune-disease-with-unusual-clinical-presentation-mimicking-systemic-lupus-erythematosus
#12
A Carbonella, G Mancano, E Gremese, F S Alkuraya, N Patel, F Gurrieri, G Ferraccioli
We describe the third family in the world, after Arabian and Turkish ones, displaying an autosomal recessive autoimmune disease (AID), mimicking systemic lupus erythematosus (SLE), with unusual manifestations due to a homozygous frame-shift variant in DNASE1L3. SLE is a complex AID characterized by multiple organ involvement. Genetic risk variants identified account for only 15% of SLE heritability. Rare Mendelian forms have been reported, including DNASE1L3-related SLE. Through specific genetic tests we identified a homozygous 2 bp-deletion c...
June 2017: Lupus
https://www.readbyqxmd.com/read/27118389/management-of-small-vessel-vasculitides
#13
REVIEW
Giuseppe Lopalco, Donato Rigante, Vincenzo Venerito, Giacomo Emmi, Maria Grazia Anelli, Giovanni Lapadula, Florenzo Iannone, Luca Cantarini
Inflammation mediated by cells of the immune system and necrosis are the most striking features observed at the histologic level in patients with vasculitides, clinical entities classified according to pathologic findings involving different organs, to etiology, or to size of vessels involved. Small vessel vasculitides (SVV) are a peculiar group of systemic disorders electively involving small intraparenchymal arteries, arterioles, capillaries, or venules and leading to different levels of vascular obstruction, tissue ischemia and risk of infarction; they can be divided into anti-neutrophil cytoplasmic antibody-associated vasculitides and immune complex vasculitides...
June 2016: Current Rheumatology Reports
https://www.readbyqxmd.com/read/26372544/-the-situation-regarding-hypocomplementemic-urticarial-vasculitis-in-2015
#14
EDITORIAL
B Flageul
No abstract text is available yet for this article.
October 2015: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/26276260/hypocomplementemic-urticarial-vasculitis-arising-in-a-patient-with-immunoglobulin-g4-related-disease
#15
Mariko Takao, Toshihisa Hamada, Tatsuya Kaji, Kazuko Ikeda-Mizuno, Chinatsu Takehara-Yasuhara, Kouichi Ichimura, Hiroyuki Yanai, Tadashi Yshino, Keiji Iwatsuki
BACKGROUND: Hypocomplementemic urticarial vasculitis (HUV) has been defined as a syndrome associated with urticarial lesions caused by leukocytoclastic vasculitis. It has been observed in patients with systemic lupus erythematosus and related conditions. Immunoglobulin G4 (IgG4)-related disease is a lymphoproliferative disorder characterized by sclerosing pancreatitis, retroperitoneal fibrosis, sclerotic cholangitis, acute interstitial nephritis, and Mikulicz's disease, and associated with elevated levels of IgG4 and hypocomplementemia...
April 2016: International Journal of Dermatology
https://www.readbyqxmd.com/read/26203836/hypocomplementemic-urticarial-vasculitis-syndrome-possibly-secondary-to-etanercept-use
#16
LETTER
Angela Wallace Fadahunsi, Melinda Garcia-Rosell, Debendra Pattanaik
No abstract text is available yet for this article.
August 2015: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/25613197/-hypocomplementaemic-urticarial-vasculitis-with-bullous-lesions-and-pericardial-involvement
#17
T Kervarrec, R Binois, C Bléchet, É Estève
BACKGROUND: Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare disease involving urticarial cutaneous vasculitis, hypocomplementaemia and systemic manifestations. Pericardial involvement occurs in very rare cases. We report a case of HUVS associated with specific pericarditis and bullous lesions. PATIENTS AND METHODS: A 63-year-old woman consulted for chronic urticaria that had appeared ten months earlier. Her skin lesions were associated with weight loss of 10 kg, deterioration of respiratory function and abdominal pain...
October 2015: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/25516220/vasculitides-with-cutaneous-expression-in-children-clinico-pathological-correlations
#18
G Borroni, S Grassi, A Carugno
The most recent pediatric vasculitis classifications (EULAR/PRINTO/PRES) have proposed the use of an integration of clinical signs and symptoms, laboratory data, imaging and pathologic data. Pediatric vasculitis represent a peculiar clinical-diagnostic model, compared to the corresponding adult pathology chapter, and in particular, dermatopathologic aspects of these diseases identify more specific issues, made contingent by crucial variables such as duration of vasculitis lesion, site of the biopsy, proper biopsy depth, and possibility to correlate histopathological findings with immunopathological results...
February 2015: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/25433930/-urticarial-vasculitis-associated-with-essential-thrombocythaemia-progressing-to-myelofibrosis
#19
C Koudoukpo, M Jachiet, J-M Zini, A Andreoli, L Pinquier, M Rybojad, D Bosset, A de Masson, M Bagot, C Lebbé, J-D Bouaziz
BACKGROUND: Urticarial vasculitis (UV) is a rare form of leukocytoclastic vasculitis in which skin lesions resemble urticaria. UV comprises hypocomplementemic and normocomplementemic subtypes. To date, only 4 cases of UV associated with myeloproliferative disorders have been described, including 3 cases with essential thrombocythaemia (ET) and one case with polycythaemia vera. PATIENTS AND METHODS: We describe the case of a 59-year-old male patient with JAK2-positive TE and secondary myelofibrosis and who developed multiple urticarial papules persisting for more than 24hours...
December 2014: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/25385679/the-clinical-spectrum-and-therapeutic-management-of-hypocomplementemic-urticarial-vasculitis-data-from-a-french-nationwide-study-of-fifty-seven-patients
#20
Marie Jachiet, Béatrice Flageul, Alban Deroux, Alain Le Quellec, François Maurier, Florence Cordoliani, Pascal Godmer, Claire Abasq, Leonardo Astudillo, Pauline Belenotti, Didier Bessis, Adrien Bigot, Marie-Sylvie Doutre, Mikaël Ebbo, Isabelle Guichard, Eric Hachulla, Emmanuel Héron, Géraldine Jeudy, Noémie Jourde-Chiche, Denis Jullien, Christian Lavigne, Laurent Machet, Marie-Alice Macher, Clotilde Martel, Sara Melboucy-Belkhir, Cécile Morice, Antoine Petit, Bernard Simorre, Thierry Zenone, Laurence Bouillet, Martine Bagot, Véronique Frémeaux-Bacchi, Loïc Guillevin, Luc Mouthon, Nicolas Dupin, Selim Aractingi, Benjamin Terrier
OBJECTIVE: Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology that is rarely described in the literature. We undertook this study to analyze the clinical spectrum and the therapeutic management of patients with HUV. METHODS: We conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histologic leukocytoclastic vasculitis, and hypocomplementemia. We assessed clinical and laboratory data and evaluated the patients' cutaneous and immunologic responses to therapy...
February 2015: Arthritis & Rheumatology
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