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urticarial vasculitis

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
A Escudier, F-X Mauvais, P Bastard, C Boussard, A Jaoui, V Koskas, E Lecoq, A Michel, M-C Orcel, P-E Truelle, D Wohrer, M Piram
Auto-inflammatory diseases are characterized by unexplained and recurrent attacks of systemic inflammation often involving the skin, joints, or serosal membranes. They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis...
January 29, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Silvia Alberti-Violetti, Emilio Berti, Angelo V Marzano
Cutaneous vasculitides encompass a wide and heterogeneous group of diseases affecting skin blood vessels that are clinically characterized by polymorphic skin lesions, including in particular palpable purpura as well as urticarial and necrotic-ulcerative lesions, with possible, albeit rare, extracutaneous involvement. Cutaneous leukocytoclastic angiitis and urticarial vasculitis, which are the two prototypic and most common variants of this group, are usually idiopathic but may also be induced by different triggers, notably drugs and infections, or may manifest in association with systemic disorders, particularly lupus erythematosus...
January 24, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
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
Virginia Velasco-Tamariz, Marta Prieto-Barrios, Fátima Tous-Romero, Sara Isabel Palencia-Pérez, Concepción Postigo-Llorente
The first vaccine that shows significant potential in providing broad coverage against serogroup B meningococcal disease has recently been approved. Because of its newness, potential adverse events need to be reported. Here we report a case of urticarial vasculitis, a rare disease in children, in probable relationship with the novel vaccine.
November 21, 2017: Pediatric Dermatology
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
Alexios Alexopoulos, Maria Dakoutrou, Kalliopi Stefanaki, George Chrousos, Talia Kakourou
BACKGROUND: Existing studies of children with vasculitis are limited. The aim of this study was to assess the epidemiology, clinical manifestations, laboratory findings, course, and outcome of Greek children presenting with vasculitic rash. METHODS: The relevant data included in the study were collected retrospectively using a standardized form from children who were admitted into our department between 2003 and 2013, with the provisional diagnosis of vasculitis of the skin...
November 2017: International Journal of Dermatology
Laure Frumholtz, Sara Laurent-Roussel, Olivier Aumaître, François Maurier, Guillaume Le Guenno, Agnes Carlotti, Alexiane Dallot, Jean Louis Kemeny, Laurent Antunes, Nicolas Froment, Sylvie Fraitag, Jonathan London, Alice Berezne, Benoît Terris, Claire Le Jeunne, Luc Mouthon, Selim Aractingi, Loïc Guillevin, Nicolas Dupin, Benjamin Terrier
OBJECTIVES: Cutaneous manifestations (CM) in ANCA-associated vasculitides (AAV) are frequent, but data on clinical significance and clinical-pathological correlations are lacking. METHODS: We conducted a multicenter, retrospective study including 1553 AAV patients. Clinical, biological and pathological features have been analyzed, and tissue samples from 46 biopsies were reviewed in a blind manner. RESULTS: CM were more frequent in EGPA (53...
November 2017: Autoimmunity Reviews
Julie K Kim, Daniel Har, L Steven Brown, David A Khan
BACKGROUND: Chronic urticaria (CU) is urticaria that has been present continuously or intermittently for at least 6 weeks. Although the prevalence and characteristics of CU are well established, little is known about recurrent CU (RCU). OBJECTIVES: We sought to establish a definition, determine the frequency, and evaluate risk factors for RCU. METHODS: A retrospective chart review of adult patients with CU evaluated at the University of Texas Southwestern allergy and immunology clinic was performed...
September 6, 2017: Journal of Allergy and Clinical Immunology in Practice
Aviv Barzilai, Lior Sagi, Sharon Baum, Henri Trau, Michael Schvimer, Iris Barshack, Michal Solomon
BACKGROUND: The classic histopathological findings of urticaria include dermal edema and a sparse perivascular infiltrate of neutrophils, eosinophils, macrophages, and lymphocytes. However, this pattern is inconsistently described. OBJECTIVE: To describe the histological and immunofluorescence characteristics of urticaria and to identify distinctive patterns. METHODS: A retrospective study was performed in which the medical files and biopsy specimens of 58 patients with acute and chronic classical urticaria were reviewed...
October 2017: American Journal of Dermatopathology
Sebastiano A G Lava, Gregorio P Milani, Emilio F Fossali, Giacomo D Simonetti, Carlo Agostoni, Mario G Bianchetti
In childhood, cutaneous small-vessel vasculitides include Henoch-Schönlein syndrome, a systemic vasculitis, and Finkelstein-Seidlmayer syndrome, a skin-limited vasculitis. Both Henoch-Schönlein and Finkelstein-Seidlmayer syndromes are seen more frequently in white or Asian compared with black children and occur especially in winter and spring with a male-to-female ratio of approximately 2:1. In everyday clinical practice, both conditions are diagnosed on clinical grounds without histological confirmation...
December 2017: Clinical Reviews in Allergy & Immunology
Adrián Imbernón-Moya, Elena Vargas-Laguna, Fernando Burgos, Eva Fernández-Cogolludo, Antonio Aguilar-Martínez, Miguel Ángel Gallego-Valdés
Annular urticarial lesions in a child must establish a main differential diagnosis with urticaria multiforme, common urticaria, acute hemorrhagic edema of infancy, erythema marginatum, erythema annulare centrifugum, annular erythema in childhood, erythema multiforme, Sweet's syndrome, Schönlein-Henoch purpura, erythematosus lupus, several systemic vasculitis, and serum sickness.
August 2017: Clinical Case Reports
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
Badriya AlHermi, Zakiya Al Mosawi, Deena Mohammed
Hypocomplementic urticarial vasculitis syndrome (HUVS) is an autoimmune disease characterized by recurrent urticaria, arthritis, and glomerulonephritis (GN). Anti-C1q antibody is the marker of HUVS together with low levels of classical pathway complements which are C2, C3, C4, and C1q. We report a case of a 6-year-old boy who presented with episodes of rashes, injected conjunctiva, abdominal pain, and arthritis, diagnosed as HUVS. He had low C3, low CH50, normal C4, and positive C1q antibody. His urinalysis showed intermittent microscopic hematuria only...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
Miyuki Fukunaga, Kazutoshi Harada, Kenichiro Mae, Kanae Wakamatsu, Noriko Kiriyama, Ryoji Tsuboi, Yukari Okubo
The etiology of purpura in Sjögren syndrome (SS) includes cryoglobulinemia, hypergammaglobulinemia, and leukocytoclastic vasculitis (LCV). The clinical symptoms of LCV associated with SS comprise palpable or nonpalpable purpura and urticarial vasculitis. Here, we report a case of LCV presenting as erythema gyratum repens (EGR)-like purpura in a 62-year-old woman with rheumatoid arthritis and SS. EGR-like skin lesions, characterized by annular lesions with expanding concentric pattern and coalescing to form a zebra-like pattern or grain of wood pattern, can appear in various autoimmune conditions; however, EGR-like eruption in SS is extremely rare...
May 2017: Case Reports in Dermatology
Howard E Gendelman, Yuning Zhang, Pamela Santamaria, Katherine E Olson, Charles R Schutt, Danish Bhatti, Bhagya Laxmi Dyavar Shetty, Yaman Lu, Katherine A Estes, David G Standaert, Elizabeth Heinrichs-Graham, LuAnn Larson, Jane L Meza, Matthew Follett, Erica Forsberg, Gary Siuzdak, Tony W Wilson, Carolyn Peterson, R Lee Mosley
A potential therapeutic role for immune transformation in Parkinson's disease evolves from more than a decade of animal investigations demonstrating regulatory T cell (Treg) nigrostriatal neuroprotection. To bridge these results to human disease, we conducted a randomized, placebo-controlled double-blind phase 1 trial with a well-studied immune modulator, sargramostim (granulocyte-macrophage colony-stimulating factor). We enrolled 17 age-matched non-Parkinsonian subjects as non-treated controls and 20 Parkinson's disease patients...
2017: NPJ Parkinson's Disease
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
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