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Hypocomplementemic urticarial vasculitis

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...
November 7, 2016: 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
Alenka Vizjak, Jelka Lindič, Jerica Mraz, Dušan Ferluga
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare immune-complex small vessel systemic vasculitis involving kidney in about 50% of patients. Characteristic clinical features are recurrent urticaria, decreased serum complement, skin vasculitis, arthritis, eye inflammation, abdominal pain, glomerulonephritis and positive anti-C1q antibodies. We report a 36-year-old woman with a 12-year history of recurrent urticaria and 2 years of urticarial vasculitis. On admission, she also had arthritis, pleuritis and pericarditis, abdominal pain, pathologic urinalysis, low serum values of C3, C4 and CH50...
October 2014: Pathology
Sara Melboucy-Belkhir, Sylvain Glitho, Frédéric Caux, Arsène Mekinian, Olivier Fain
No abstract text is available yet for this article.
January 2014: La Revue du Praticien
Teresa Pinto-Almeida, Mónica Caetano, Rosário Alves, Manuela Selores
Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia...
November 2013: Anais Brasileiros de Dermatologia
Zakiya Saleh Adnan Al Mosawi, Badriya Ebrahim Ahmed Al Hermi
Hypocomplementemic urticarial vasculitis syndrome is an immune complex-mediated disease of unknown etiology. The clinical course is characterized by urticaria, conjunctivitis, joint pain, and hypocomplementemia. We here report a case of a child with hypocomplementemic urticarial vasculitis syndrome that progressed to nephritis. Renal biopsy was consistent with diffuse proliferative glomerulonephritis with diffuse subendothelial immune deposits. He responded well to a combination of steroid and mofetil micofenolate...
July 2013: Oman Medical Journal
Z Birsin Ozçakar, Joseph Foster, Oscar Diaz-Horta, Ozgur Kasapcopur, Yao-Shan Fan, Fatoş Yalçınkaya, Mustafa Tekin
OBJECTIVE: Hypocomplementemic urticarial vasculitis syndrome (HUVS) is characterized by recurrent urticaria along with dermal vasculitis, arthritis, and glomerulonephritis. Systemic lupus erythematosus (SLE) develops in >50% of patients with HUVS, although the pathogenesis is unknown. The aim of this study was to identify the causative DNA mutations in 2 families with autosomal-recessive HUVS, in order to reveal the pathogenesis and facilitate the laboratory diagnosis. METHODS: Autozygosity mapping was combined with whole-exome sequencing...
August 2013: Arthritis and Rheumatism
L Breda, M Nozzi, S Harari, M Del Torto, M Lucantoni, A Scardapane, F Chiarelli
No abstract text is available yet for this article.
July 2013: Journal of Clinical Immunology
Julie M Jones, Keith A Reich, Deena G Raval
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a type III hypersensitivity reaction characterized by urticaria with persistent acquired hypocomplementemia. Although HUVS is uncommon, it is important for physicians to be familiar with this disease, as the initial presentation is often life-threatening angioedema. The author reports the case of a 47-year-old white woman with a history of HUVS. She presented to an outpatient clinic complaining of a rash and difficulty swallowing. Urticaria and angioedema were diagnosed...
February 2012: Journal of the American Osteopathic Association
Andrew Buck, Jim Christensen, Morgan McCarty
Hypocomplementemic urticarial vasculitis syndrome, as opposed to urticarial vasculitis or urticarial vasculitis syndrome, is a rare disease process where the exact pathophysiology remains unknown. This article discusses the case of a 34-year-old Hispanic man with an ongoing history of chronic urticaria comprising episodes induced by low ambient temperatures, emotional stress, and spontaneous occurrences. This article serves as a consolidated reference for specialists to comprehensively review the plethora of systemic manifestations that may accompany urticarial vasculitis and highlights new systemic complications reported in association with this disease which are also observed in this case...
January 2012: Journal of Clinical and Aesthetic Dermatology
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