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https://www.readbyqxmd.com/read/29332707/cutaneous-hemophagocytosis-clinicopathologic-features-of-21-cases
#1
Francesca Boggio, Viviana Lora, Carlo Cota, Amanda Pereira, Robert Müllegger, Lucia Prieto-Torres, Lorenzo Cerroni
BACKGROUND: Hemophagocytosis is well known in cytotoxic cutaneous T-cell lymphomas (CTCLs), in which it may represent a sign of hemophagocytic lymphohistiocytosis syndrome (HLHS), and is also typical of cutaneous Rosai-Dorfman disease (cRDD) (without prognostic relevance). Only rarely, has cutaneous hemophagocytosis (CH) been described in other skin conditions. OBJECTIVE: To characterize the clinicopathologic features of CH in skin biopsy specimens from patients with conditions other than CTCL or cRDD...
February 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29326535/very-rare-presentation-of-cerebrovascular-accident-in-20-year-old-man-with-familial-mediterranean-fever-case-report
#2
Miramir Aghdashi, Seyed-Mostafa Seidmardani, Sara Vossoughian, Seyed Arman Seyed Mokhtari
Familial Mediterranean fever (FMF) is characterized by recurrent episodes of fever accompanied by serosal, synovial, or cutaneous inflammation. The central nervous system (CNS) is rarely involved in FMF. The CNS involvement includes demyelinating lesions, posterior reversible encephalopathy syndrome, pseudotumor cerebri, optic neuritis, and cerebral vasculitis. Here, we present a 20-year-old man, a known case of FMF with abrupt left-sided hemiparesis. Brain magnetic resonance imaging revealed right periventricular infarction...
2018: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29287933/-humoral-immune-diseases-cutaneous-vasculitis-and-auto-immune-bullous-dermatoses
#3
Janine Wechsler
Humoral immunity is the cause of multiple diseases related to antibodies (IgA, IgG, IgM) produced by the patient. Two groups of diseases are identified. The first group is related to circulating antigen-antibody complexes. The antigens are various. They are often unknown. These immune complexes cause a vascular inflammation due to the complement fixation. Consequently, this group is dominated by inflammatory vasculitis. In the second group, the pathology is due to the fixation in situ of antibodies to a target antigen of the skin that is no more recognized by the patient...
December 26, 2017: Annales de Pathologie
https://www.readbyqxmd.com/read/29274703/clinical-significance-of-neutrophil-lymphocyte-ratio-in-patients-with-granulomatosis-with-polyangiitis
#4
Nouran M Abaza, Eiman M Abd El-Latif, Tamer A Gheita
OBJECTIVE: To determine to neutrophil-to-lymphocyte ratio (NLR) in granulomatosis with polyangiitis (GPA) patients and to study its relation to disease manifestations and activity. METHODS: The study included 44 GPA patients and 44 matched age and sex controls. Full history taking, thorough clinical examination with more attention to ocular examination, laboratory and radiological investigations were considered. Disease activity was assessed using the Birmingham Vasculitis Activity Score (BVAS)...
December 20, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/29273180/-multiple-facets-of-ada2-deficiency-vasculitis-auto-inflammatory-disease-and-immunodeficiency-a-literature-review-of-135-cases-from-literature
#5
A Fayand, G Sarrabay, A Belot, V Hentgen, I Kone-Paut, G Grateau, I Melki, S Georgin-Lavialle
Deficiency of adenosine deaminase 2 (DADA2) is a recently described auto-inflammatory disorder. It is an autosomal recessive inherited disease, caused by mutations in the ADA2 gene (formerly known as CECR1) encoding ADA2 enzyme. Besides its role in the purine metabolism, it has been postulated that ADA2 may act as a growth factor for endothelial cells and in the differenciation of monocytes. Thus, deficiency of ADA2 would lead to endothelial damage and a skewing of monocytes into M1 pro-inflammatory macrophage, causing DADA2 manifestations...
December 19, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29245283/successful-treatment-of-erythema-induratum-with-topical-application-of-antituberculous-drugs-a-case-report
#6
Xueling Mei, Junying Zhao
RATIONALE: Erythema induratum, a chronic recurrent lobular panniculitis with vasculitis, is strongly associated with Mycobacterium tuberculosis infection. The recommended drugs include isoniazid, rifampicin, and pyrazinamide, which are typically administered in combination (orally or intravenously). Till date, there are no reports about topical application of anti-tuberculous (anti-TB) drugs for treatment of erythema induratum. PATIENT CONCERNS: Herein, we present the case of a 73-year-old woman with recurrent ulceration, scarring and obvious pain in her lower legs...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29211697/cocaine-levamisole-associated-autoimmune-syndrome-a-disease-of-neutrophil-mediated-autoimmunity
#7
Michael J Cascio, Kuang-Yu Jen
PURPOSE OF REVIEW: Levamisole was previously used for its immunomodulatory properties to treat rheumatoid arthritis and some cancers. However, because of serious side-effects, it was taken off the market in the United States. Recently, levamisole has reemerged as a popular cocaine adulterant. Some individuals who consume levamisole-adulterated cocaine can develop a life-threatening autoimmune syndrome. In this review, the medical consequences of levamisole exposure and postulated mechanisms by which levamisole induces these adverse effects are discussed...
January 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29191345/vasculitis-kids-are-not-just-little-people
#8
Nikita Lakdawala, Flavia Fedeles
Cutaneous vasculitis, inflammatory destruction of blood vessels, can present with a wide range of clinical and pathologic findings across a number of heterogeneous conditions. Although some vasculitides are present in both children and adults, some important differences exist in clinical presentation, etiology, management, and prognosis in childhood vasculitis versus adult vasculitis. Cutaneous vasculitis is rare in children, and most childhood vasculitides, of which Henoch-Schönlein purpura is the most common, histologically are small vessel leukocytoclastic vasculitis...
November 2017: Clinics in Dermatology
https://www.readbyqxmd.com/read/29172983/a-comparison-of-brand-and-biosimilar-granulocyte-colony-stimulating-factors-for-prophylaxis-of-chemotherapy-induced-febrile-neutropenia
#9
Andrea G Douglas, Phil Schwab, Daniel Lane, Kenneth Kennedy, S Lane Slabaugh, Andy Bowe
BACKGROUND: Filgrastim-sndz, a granulocyte-colony stimulating factor (G-CSF), was introduced as a biosimilar to filgrastim in 2015, but real-world comparative effectiveness for filgrastim versus filgrastim-sndz has not been reported to date. OBJECTIVES: To (a) compare the incidence of febrile neutropenia for patients taking filgrastim versus those taking filgrastim-sndz and (b) compare the incidence of a potential serious adverse event for filgrastim versus filgrastim-sndz...
December 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/29146054/lymphocytic-thrombophilic-arteritis-a-distinct-inflammatory-type-i-interferon-and-c5b-9-mediated-subcutaneous-endovasculitis
#10
Cynthia M Magro, Jad Saab
BACKGROUND: Lymphocytic thrombophilic arteritis is a recently recognized subcuticular larger vessel vasculitis characterized by striking vascular luminal thrombosis. METHODS: The clinical features, histopathology and phenotypic profile of ten patients with lymphocytic thrombophilic arteritis were explored in an attempt to better define the entity from a clinical and pathophysiologic perspective. RESULTS: The patients were all female (mean age of 43) presenting with generally asymptomatic lower and upper extremity hyperpigmented macules...
December 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/29136340/dermatological-addendum-to-the-2012-international-chapel-hill-consensus-conference-nomenclature-of-vasculitides
#11
Cord H Sunderkötter, Bernhard Zelger, Ko-Ron Chen, Luis Requena, Warren Piette, J Andrew Carlson, Jan Dutz, Peter Lamprecht, Alfred Mahr, Elisabeth Aberer, Victoria P Werth, David A Wetter, Seiji Kawana, Raashid Luqmani, Camille Frances, Joseph Jorizzo, J Richard Watts, Dieter Metze, Marzia Caproni, Erkan Alpsoy, Jeffrey P Callen, David Fiorentino, Peter A Merkel, Ronald J Falk, J Charles Jennette
OBJECTIVE: The objective of this project was to prepare a dermatologic addendum to the 2012 Chapel Hill consensus conference nomenclature to address vasculitides affecting the skin. The goal was to standardize names and definitions for cutaneous vasculitis. METHODS: A nominal group technique with a facilitator was used to reach a consensus using multiple face-to-face meetings, e-mail discussions, and teleconferences. RESULTS: Standardized names, definitions and descriptions are provided for cutaneous components of systemic vasculitides (e...
November 14, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29128969/in-vasculitis-of-small-muscular-arteries-activation-of-vessel-infiltrating-cd8-t-cells-seems-to-be-antigen-independent
#12
Mikiko Kobayashi, Eisaku Ogawa, Ryuhei Okuyama, Hiroyuki Kanno
The etiology of polyarteritis nodosa (PAN) and localized PAN is still unknown, although a T cell-mediated immune mechanism has been considered. CD8 T cells participate not only in the antigen-dependent adaptive immune system, but also in the antigen-independent innate immune system. Non-antigen-activated CD8 T cells express a unique phenotype: granzyme B (GrB) positive /CD25 negative /programmed death-1 (PD-1) negative. The aims of this study were to assess the participation of T cells, especially innate CD8 T cells, in the development of vasculitis...
November 11, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29119480/rheumatoid-factor-false-positivity-in-patients-with-anca-associated-vasculitis-not-having-medical-conditions-producing-rheumatoid-factor
#13
Jae-Seung Moon, Diane Da-Hyun Lee, Yong-Beom Park, Sang-Won Lee
We investigated the rate of rheumatoid factor (RF) false positivity at diagnosis and its influence on clinical and prognostic features and rheumatoid arthritis (RA) development during the follow-up in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients without RA or other medical conditions triggering RF false positivity. We reviewed the medical records of 128 AAV patients. We divided patients with AAV and each variant into two groups according to RF positivity and compared variables between the two groups...
November 8, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29119304/is-thrombocytosis-always-an-indicator-of-autosplenectomy-in-patients-with-systemic-lupus-erythematosus
#14
Döndü Üsküdar Cansu, Hava Üsküdar Teke, Ahmet Musmul, Cengiz Korkmaz
In systemic lupus erythematosus (SLE), the most commonly encountered finding related to platelets is thrombocytopenia whereas thrombocytosis is rarely reported. Our aim here was to reveal the type and the frequency of thrombocytosis in SLE patients along with its causes. Data of patients were evaluated retrospectively. Patients who had a platelet count of > 450,000/mm3 (> 450 × 109/L) in at least two subsequent counts and lasting more than 6 months during the follow-up were considered to have "persistent thrombocytosis"...
November 8, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29117463/vasculitis-and-iga-monoclonal-gammopathy-of-cutaneous-significance
#15
L Rousset, F Cordoliani, M Battistella, M Jachiet, E Pillebout, M Rybojad, M Bagot, E Oksenhendler, J-D Bouaziz
IgA vasculitis (Henoch-Schonlein purpura) is a small vessel systemic vasculitis involving IgA immunoglobulin polyclonal activation(1) . Monoclonal gammopathy of cutaneous significance is a subgroup of monoclonal gammopathy with skin disease, without myeloma or lymphoproliferative disorder(2) . We report 3 patients with vasculitis and IgA monoclonal gammopathy of cutaneous significance. Three men, 38, 37 and 50 years old, had a vascular purpura for more than 10 years with partial remission and frequent necrotic flares (Figure 1)...
November 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29115092/hypocomplementemic-urticarial-vasculitis-syndrome-with-membranous-nephropathy-case-report
#16
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/29097818/dapsone-treatment-is-efficient-against-persistent-cutaneous-and-gastrointestinal-symptoms-in-children-with-henoch-sch%C3%A3-nlein-purpura
#17
Jana Volejnikova, Jaroslav Horacek, Frantisek Kopriva
BACKGROUND: Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and by the deposition of IgA immune complexes. An association between HSP and atypical bacteria is uncommon in children. METHODS AND RESULTS: Here we report three cases of children, aged 5, 4 and 16 years, who were diagnosed with HSP associated with Mycoplasma pneumoniae or Chlamydia pneumoniae infection. In all presented cases, persistent cutaneous manifestations and abdominal pain were resistant to antibiotics and corticosteroids, but resolved during 48 h after the introduction of dapsone...
November 2, 2017: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/29068068/late-stage-nodular-erythema-elevatum-diutinum-mimicking-sclerotic-fibroma
#18
Kevin Y Shi, Travis Vandergriff
Erythema elevatum diutinum (EED) is a rare, cutaneous vasculitis of uncertain origin. EED can present clinically as chronic bilateral, symmetrical, periarticular papules, plaques and nodules. We report here an unusual case of EED presenting as multiple, densely fibrosing nodules on the feet of a 60-year-old human immunodeficiency virus positive woman. The initial evaluation of the patient was complicated by the strong histologic resemblance of multiple lesions to sclerotic fibroma, a cutaneous manifestation of Cowden disease...
January 2018: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/29057130/cutaneous-vasculitis-an-unusual-presentation-of-a-biclonal-nodal-plasma-cell-dyscrasia
#19
D Swan, M Murphy, E Elhassadi
We describe an unusual case of a biclonal nodal plasma cell dyscrasia, presenting with a vasculitic rash, end-organ damage, and cytopenias. Serum protein electrophoresis demonstrated a biclonal kappa-restricted paraprotein, with a negative skeletal survey and no bone marrow disease. Fluorodeoxyglucose-PET-CT (FDG-PET-CT) revealed nodal involvement, which was not appreciable clinically, and facilitated biopsy, confirming the diagnosis of a nodal plasmacytoma. Complete biochemical response and resolution of the vasculitic rash were achieved with bortezomib-based therapy...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29050884/initial-digital-vasculitis-in-a-large-multicenter-cohort-of-childhood-onset-systemic-lupus-erythematosus
#20
Ana Paula Sakamoto, Clovis Artur Silva, Marco Felipe Castro da Silva, Anandreia Simões Lopes, Gleice Clemente Souza Russo, Adriana Maluf Elias Sallum, Katia Kozu, Eloisa Bonfá, Claudia Saad-Magalhães, Rosa Maria Rodrigues Pereira, Claudio Arnaldo Len, Maria Teresa Terreri
OBJECTIVES: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. METHODS: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. RESULTS: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%)...
October 16, 2017: Revista Brasileira de Reumatologia
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