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https://www.readbyqxmd.com/read/28633733/rituximab-in-the-management-of-juvenile-pemphigus-foliaceus
#1
Tiffany Y Loh, Taraneh Paravar
BackgroundPemphigus foliaceus (PF) is a blistering disorder most commonly presenting in middle age. As PF is restricted to the superficial epidermis, it is considered more benign than other pemphigus diseases. However, progression to severe disease is not uncommon. Although rituximab's efficacy has been well-documented in adults with refractory PF, little data is available on its role in adolescents.PurposeWe describe a patient with juvenile PF treated with rituximab and review the literature for similar cases...
June 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28632473/a-rare-occurrence-of-epidermolysis-bullosa-acquisita-in-a-patient-with-retroviral-disease
#2
Zambare Uddhao, Chhonkar Aditi, Nayak Chitra, Tambe Swagata
Epidermolysis bullosa acquisita is a chronic subepidermal blistering disease associated with autoimmunity to type-VII collagen within anchoring fibrils located at the dermo-epidermal junction. This entity is rarely reported from India. It can have a variety of presentations. Its occurrence with retroviral disease has rarely been reported. Here, we present a case of epidermolysis bullosa acquisita in a patient with HIV infection.
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28630891/frequency-of-neurological-disorders-in-bullous-pemphigoid-patients-a-cross-sectional-study
#3
Sheida Khosravani, Farhad Handjani, Reyhaneh Alimohammadi, Nasrin Saki
BACKGROUND: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disorder which occurs mostly in the elderly. Several studies have reported an association between BP and neurological disorders (ND). OBJECTIVE: The purpose of this study was to evaluate the association between BP and neurological disorders in Iranian patients. METHODS: In this cross-sectional study, 87 patients with BP were enrolled. They were compared to 184 controls...
2017: International Scholarly Research Notices
https://www.readbyqxmd.com/read/28623863/linear-immunoglobulin-a-g-bullous-dermatosis-associated-with-ulcerative-colitis
#4
Asuka Onoe, Daisuke Matsuura, Tadashi Terui, Norito Ishii, Takashi Hashimoto, Toyoko Ochiai
Linear immunoglobulin (Ig)A/G bullous dermatosis (LAGBD) is an autoimmune bullous disease characterized by formation of subepidermal blisters and linear deposition of IgA and IgG antibodies along the basement membrane zone (BMZ). The association between linear IgA bullous dermatosis and ulcerative colitis (UC) is well recognized, but reports of UC-associated LAGBD are lacking. We have reported a 24-year-old man suffering from LAGBD associated with UC, which occurred before exacerbations of skin rash. A skin biopsy indicated a subepidermal blister with an infiltration of primarily neutrophils and eosinophils in the dermis...
June 17, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28620161/different-signaling-patterns-contribute-to-loss-of-keratinocyte-cohesion-dependent-on-autoantibody-profile-in-pemphigus
#5
Elias Walter, Franziska Vielmuth, Lukas Rotkopf, Miklós Sárdy, Orsolya N Horváth, Matthias Goebeler, Enno Schmidt, Rüdiger Eming, Michael Hertl, Volker Spindler, Jens Waschke
Pemphigus is an autoimmune blistering skin disease caused primarily by autoantibodies against desmoglein (Dsg)1 and 3. Here, we characterized the mechanisms engaged by pemphigus IgG from patients with different clinical phenotypes and autoantibody profiles. All pemphigus vulgaris (PV) and pemphigus foliaceus (PF) IgG and AK23, a monoclonal mouse antibody against Dsg3, caused loss of cell cohesion, cytokeratin retraction and p38MAPK activation. Strong alterations in Dsg3 distribution were caused by mucosal (aDsg3 antibodies), mucocutaneous (aDsg1 + aDsg3) as well as atypical (aDsg3) PV-IgG...
June 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28615054/end-stage-kidney-disease-in-patient-with-epidermolysis-bullosa-what-are-the-treatment-options-case-report
#6
Michał Małecki, Maciej Domański, Kazimierz Ciechanowski
BACKGROUND: Epidermolysis bullosa is a group of diseases caused by mutations in genes for proteins responsible for cells' anchorage at the dermo-epidermal junction. Their common feature are dysfunctional or even absent connections between cells. The typical clinical sign is the formation of blisters, with possible excessive scarring, in response to minimal skin irritation. End stage renal disease may be one of the comorbidities in patients with epidermolysis bullosa. The implementation of renal replacement therapy may be very difficult in this population...
June 14, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28614800/robust-memory-responses-against-influenza-vaccination-in-pemphigus-patients-previously-treated-with-rituximab
#7
Alice Cho, Bridget Bradley, Robert Kauffman, Lalita Priyamvada, Yevgeniy Kovalenkov, Ron Feldman, Jens Wrammert
Rituximab is a therapeutic anti-CD20 monoclonal antibody widely used to treat B cell lymphoma and autoimmune diseases, such as rheumatic arthritis, systemic lupus erythematosus, and autoimmune blistering skin diseases (AIBD). While rituximab fully depletes peripheral blood B cells, it remains unclear whether some preexisting B cell memory to pathogens or vaccines may survive depletion, especially in lymphoid tissues, and if these memory B cells can undergo homeostatic expansion during recovery from depletion...
June 15, 2017: JCI Insight
https://www.readbyqxmd.com/read/28611508/correlation-between-il36%C3%AE-and-il17-and-activity-of-the-disease-in-selected-autoimmune-blistering-diseases
#8
Agnieszka Żebrowska, Anna Woźniacka, Katarzyna Juczyńska, Kamila Ociepa, Elżbieta Waszczykowska, Izabela Szymczak, Rafał Pawliczak
Dermatitis herpetiformis (DH), bullous pemphigoid (BP), and pemphigus vulgaris (PV) are autoimmune bullous skin conditions with eosinophilic and neutrophilic infiltrations. While cytokines are crucial for the affinity and activation of different leukocyte cells in the inflammation and blister formation, there are no studies concerning a role of IL-36. The goal of the study was to analyze whether interleukin 36 is involved in pathogenesis of DH, BP, and PV. And the second aim of the study was the estimation of correlation between Il-36 and IL-17 and titers of specific antibodies in these diseases...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28600798/inhibition-of-p38mapk-signaling-prevents-epidermal-blistering-and-alterations-of-desmosome-structure-induced-by-pemphigus-autoantibodies-in-human-epidermis
#9
D T Egu, E Walter, V Spindler, J Waschke
BACKGROUND: Pemphigus vulgaris (PV) is a skin blistering disease caused by autoantibodies targeting the desmosomal adhesion proteins desmoglein (Dsg) 3 and 1. The mechanisms underlying pemphigus skin blistering are not fully elucidated but p38MAPK activation is one of the signaling events necessary for full loss of cell cohesion. However, it is unclear whether ultrastructural hallmarks of desmosome morphology as observed in patients' lesions are mediated by p38MAPK signaling. OBJECTIVE: In this study, we tested the relevance of p38MAPK for blister formation and the ultrastructural changes induced by PV autoantibodies in human skin...
June 10, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28599695/junctional-epidermolysis-bullosa-non-herlitz-type
#10
Munir Ahmad Bhinder, Muhammad Waqar Arshad, Muhammad Yasir Zahoor, Wasim Shehzad, Muhammad Tariq, Muhammad Imran Shabbir
Junctional epidermolysis bullosa (JEB) is a recessively inherited skin blistering disease and is caused due to abnormalities in proteins that hold layers of the skin. Herlitz JEB is the severe form and non-Herlitz JEB is the milder form. This report describes a case of congenitally affected male child aged 5 years, with skin blistering. He has mitten-like hands and soft skin blistering on hands, legs and knees. Symptoms almost disappeared at the age of 3 years but reappeared with increased severity after 6 months...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28592740/serum-amyloid-a-protein-in-blistering-skin-disease-and-peripheral-circulatory-disturbance
#11
(no author information available yet)
No abstract text is available yet for this article.
2017: Nihon Ronen Igakkai Zasshi. Japanese Journal of Geriatrics
https://www.readbyqxmd.com/read/28590036/t-regulatory-cells-and-other-lymphocyte-subsets-in-patients-with-bullous-pemphigoid
#12
T Gambichler, A Tsitlakidon, M Skrygan, S Höxtermann, L Susok, S Hessam
BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disease, and is associated with autoantibodies to the hemidesmosomal BP autoantigens BPAG1 and BPAG2. AIM: We aimed to investigate the significance of T regulatory cells and other lymphocyte subsets in patients with BP. METHODS: In total, 31 inpatients with BP were treated with systemic prednisolone in a tapered dose regimen, while 28 healthy individuals matched for age and sex served as the healthy control (HC) group...
June 7, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28584373/prospective-analysis-of-skin-findings-in-surgical-critically-ill-patients-intensive-care-unit
#13
Suzan Demir Pektas, Arzu Kahveci Demir
BACKGROUND: Intensive Care Units (ICUs) are places where critically ill patients are managed. AIM: We aimed to investigate skin disorders that developed in critically ill surgical patients during their stay in the ICU. METHODS: The prevalence of dermatological disorders and factors affecting their clinical features was prospectively analyzed in surgical ICU patients. We recorded age, sex, type of ICU, comorbidities, skin disorders, time to consultation, duration of ICU stay, and mortality rate...
May 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28584371/shifting-focus-in-the-therapeutics-of-immunobullous-disease
#14
Abhishek De, Asad Ansari, Nidhi Sharma, Aarti Sarda
Therapeutics of autoimmune bullous disease has seen a major shift of focus from more global immunosuppression to targeted immunotherapy. Anti CD 20 monoclonal antibody Rituximab revolutionized the therapeutics of autoimmune bullous disease particularly pemphigus. Though it is still being practiced off-label, evidences in the form of RCT and meta analysis are now available. Other novel anti CD 20 monoclonal antibodies like ofatumumab, veltuzumab, and ocrelizumab, tositumomab or obinutuzumab/GA101 may add to the therapeutic options in coming days...
May 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28584370/counseling-in-pemphigus
#15
Raghavendra Rao, C R Srinivas
Pemphigus is a chronic, potentially life threatening, autoimmune intraepidermal blistering disease affecting the skin and the mucous membrane. Pemphigus may have profound impact on the quality of life in the affected patients. Counselling and adjuvant psychotherapy may be beneficial at least in few patients and should be considered as a part of multidisciplinary approach.
May 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28584369/advanced-diagnostic-techniques-in-autoimmune-bullous-diseases
#16
Anuradha Jindal, Raghavendra Rao, Balbir S Bhogal
Autoimmune blistering diseases are diverse group of conditions characterized by blisters in the skin with or without mucosal lesions. There may be great degree of clinical and histopathological overlap; hence, advanced immunological tests may be necessary for more precise diagnosis of these conditions. Direct immunofluorescence microscopy is the gold standard tests to demonstrate the tissue-bound antibodies and should be done in all cases. Magnitude of antibody level in patient' serum can be assessed by indirect immunofluorescence and enzyme linked immunosorbent assay...
May 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28571819/chronic-eczematous-dermatitis-in-patients-with-neurodegenerative-diseases-may-be-an-early-marker-of-bullous-pemphigoid
#17
Ping-Song Chou, Tsai-Ching Chou, Chung-Hsing Chang, Sebastian Yu, Chih-Hung Lee
The number of elderly patients with chronic pruritus has been gradually increasing in aging countries. Bullous pemphigoid, a common autoimmune blister disease in the elderly, is always heralded by pruritic eczematous dermatitis and is often associated with neurodegenerative diseases. We hypothesized that chronic eczematous dermatitis in patients with neurodegenerative diseases may be an early marker of bullous pemphigoid. By conducting retrospective chart review, we found neurodegenerative diseases are more prevalent in elderly patients with chronic eczematous dermatitis...
June 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28567313/puva-induced-bullous-pemphigoid-in-a-patient-with-mycosis-fungoides
#18
Birgül Özkesici, Saliha Koç, Ayşe Akman-Karakaş, Ertan Yılmaz, İbrahim Cumhur Başsorgun, Soner Uzun
Background. Bullous pemphigoid is an autoimmune subepidermal blistering skin disease in which autoantibodies are directed against components of the basement membrane. The disease primarily affects the elderly people and in most of the patients inducing factors cannot be identified. Herein, we report a case of BP that occurred in a patient who was receiving PUVA therapy for the treatment of mycosis fungoides. Main Observation. A 26-year-old woman with mycosis fungoides developed blisters while receiving PUVA therapy...
2017: Case Reports in Dermatological Medicine
https://www.readbyqxmd.com/read/28559814/the-rare-case-of-pemphigus-vegetans-in-association-with-malnutrition-children-in-the-multidisciplinary-management
#19
Oki Suwarsa, Endang Sutedja, Hartati Purbo Dharmadji, Pramita Kusuma, July Rahardja, Reti Hindritiani, Hendra Gunawan
Pemphigus vegetans is a clinical variant of pemphigus vulgaris, accounting for 1-2% of all very rare pemphigus cases in children. The involvement of the oral mucosa in this disease is usually accompanied by severe pain that aggravates the patient's malnourished condition. Conversely, malnutrition may also reduce vulnerability towards autoimmune diseases. Although pemphigus vegetans has never been reported to develop in a child with marasmus before, we encountered a case of pemphigus vegetans in a severely malnourished patient...
January 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28550834/-bullous-pemphigoid-successfully-treated-with-rituximab
#20
Nuno Silva, Andreia Costa, Fernando Salvador, Elisa Serradeiro
Bullous pemphigoid is an autoimmune disease characterized by subepidermal blister formation. Can be self-limiting but when necessary treatment includes the use of corticosteroids, immunosuppressants, and biological agents. We present the case of a 37 year old patient with a severe and refractory presentation of bullous pemphigoid. After about a month of treatment with various first line drugs and with no response, we chose to start rituximab and clear improvement of the lesions was observed. The authors aim to highlight the usefulness and effectiveness of this drug in this disease...
March 31, 2017: Acta Médica Portuguesa
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