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Hypocomplementemic urticarial vasculitis

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
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
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
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
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
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
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...
April 27, 2017: La Revue de Médecine Interne
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
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
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
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
B Flageul
No abstract text is available yet for this article.
October 2015: Annales de Dermatologie et de Vénéréologie
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
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
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
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
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
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
Chuiyoung Park, Seung Won Choi, Misung Kim, Jongha Park, Jong Soo Lee, Hyun Chul Chung
INTRODUCTION: Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud's arthropathy, and valvular heart disease have been reported. CASE PRESENTATION: A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema...
October 22, 2014: Journal of Medical Case Reports
Pavel Horak, Martina Skacelova, Josef Zadrazil, Andrea Smrzova, Karel Krejcí, Hana Ciferska, Zuzana Hermanova
SLE is characterized by overproduction of various types of autoantibodies. Under certain circumstances, antibodies targeting some of the neoepitopes of the complement system can be seen. The most studied among antibodies directed against a component of the complement system is anti-C1q. Anti-C1q antibodies are present in approximately one third of the patients with lupus, who often have high clinical disease activity and in particular renal involvement. In the presence of high titers of anti-C1q antibodies also the levels of C1q and C3 and C4 components of the complement system are also usually low...
2013: Current Rheumatology Reviews
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