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Pemphigus

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https://www.readbyqxmd.com/read/28413387/successful-treatment-of-a-bullous-pemphigoid-patient-with-rituximab-who-was-refractory-to-corticosteroid-and-omalizumab-treatments
#1
Aslı Bilgiç Temel, Cumhur Ibrahim Bassorgun, Ayşe Akman-Karakaş, Erkan Alpsoy, Soner Uzun
Omalizumab is a humanized monoclonal antibody which is an FDA-approved treatment of severe allergic asthma and inhibits IgE binding to FcεRI. According to increasing evidence of IgE inhibition, omalizumab was suggested as a therapeutic approach for bullous pemphigoid (BP). Rituximab has been reported to be effective in various autoimmune diseases, including autoimmune bullous dermatoses. A specific protocol for the use of rituximab to treat BP patients is not yet available. There are only small case series and case reports about the efficacy and safety of rituximab in BP...
January 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28401573/is-tocilizumab-a-potential-therapeutic-option-for-refractory-unicentric-castleman-disease
#2
Muhammad Bilal Abid, Rachel Peck, Muhammad Abbas Abid, Wesam Al-Sakkaf, Yuening Zhang, Giles S Dunnill, Konrad Staines, Iara-Maria Sequeiros, Lisa Lowry
Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. We present a case of a patient, negative for HIV and HHV-8, who had an unsuccessful surgical intervention and no response to radiotherapy and chemotherapy...
April 11, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28400637/comparison-of-immunofluorescence-and-desmoglein-enzyme-linked-immunosorbent-assay-in-the-diagnosis-of-pemphigus-a-prospective-cross-sectional-study-in-a-tertiary-care-hospital
#3
Deepthi Ravi, S Smitha Prabhu, Raghavendra Rao, C Balachandran, Indira Bairy
BACKGROUND: Pemphigus is an acquired immunobullous disorder in which antibodies are directed against epidermal cadherins. Despite the commercial availability and less cost of enzyme-linked immunosorbent assays (ELISAs) to detect antidesmoglein 1 (Dsg1) and anti-Dsg3, immunofluorescence is still widely used for confirmation of diagnosis. AIMS: (1) To compare the usefulness of indirect immunofluorescence (IIF) and ELISA tests in the diagnosis of pemphigus. (2) To find the clinical correlation between the tests and severity of the disease...
March 2017: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/28397714/effect-of-intravenous-pulse-dexamethasone-versus-daily-oral-prednisolone-on-bone-mineral-density-in-dermatology-patients-is-it-a-site-specific-response
#4
Sanjeev Handa, Gurjeet Singh, Amanjot Kaur Arora, Niranjan Khandelwal, Vivek Gupta
BACKGROUND: The use of glucocorticoids in various forms of administration is complicated by their systemic side effects. Although intravenous pulse therapy is considered to have lesser systemic side effects, there are few studies in literature comparing the effects of intravenous pulse glucocorticoids versus oral daily glucocorticoids on bone mineral density. AIM: To compare the effects of intravenous pulse glucocorticoids and oral daily glucocorticoids on bone mineral density with the aim of finding any site-specific osteopenic side effect...
April 10, 2017: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/28395432/-clinicopathological-features-of-acantholytic-mammary-paget-s-disease-a-report-of-28-cases
#5
Y P Zeng, C Chen, K Fang, Q N Jia, D L Ma
Objective: To investigate the clinicopathological features of acantholytic mammary Paget's disease (AMPD). Methods: From January, 2010 to October, 2016, a total of 28 patients were diagnosed as AMPD in the Department of Dermatology, Peking Union Medical College Hospital. The clinical and histopathological data of these patients were analyzed retrospectively. Results: The patients were all female. The mean age of onset was (51±15)years (range, 24 to 78 years). The median course of disease was 10.5 months (range, 3 months to 2 years)...
April 11, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28394553/drug-induced-oral-ulcerations-case-report
#6
Vanja Vučičević Boras, Snježana Židovec-Lepej, Branka Marinović, Sven Seiwerth, Ivana Škrinjar, Dražen Pulanić, Danica Vidović Juras
A 70-year-old patient was admitted to the Department of Oral Medicine for multiple oral ulcerations on the left buccal mucosa, around 0.5 cm in diameter, as well as on the gingiva. Otherwise, the patient suffered from chronic lymphocytic leukemia, hypogammaglobulinemia, chronic renal insufficiency, with complete afunction of the right kidney, asthma, hypertension, gastritis and prostate hyperplasia. Differential diagnosis of oral ulcerations included drug induced oral ulcerations, paraneoplastic pemphigus, viral ulcerations (cytomegalovirus, herpes simplex viruses), fungal ulcerations (candidiasis, aspergillosis, histoplasmosis, cryptococcosis) and bacterial ulcerations, as well as neutropenic ulcers...
June 2016: Acta Clinica Croatica
https://www.readbyqxmd.com/read/28393203/increased-mir-424-5p-expression-in-peripheral-blood-mononuclear-cells-from-patients-with-pemphigus
#7
Menglei Wang, Liuping Liang, Li Li, Kai Han, Qian Li, Yusheng Peng, Xuebiao Peng, Kang Zeng
Pemphigus is an autoimmune disease that causes blisters and erosions in the skin and mucous membranes. The development of pemphigus is associated with the imbalance of T‑cell and humoral responses. MicroRNAs (miRNAs) can regulate many cell functions. However, whether miRNA expression is altered in peripheral blood mononuclear cells (PBMCs) during the pathogenesis of pemphigus has not been clarified. The aim of the present study was to examine the miRNA expression profiles of PBMCs from patients with pemphigus...
April 3, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28392652/paraneoplastic-pemphigus-associated-with-a-malignant-thymoma-a-case-of-persistent-and-refractory-oral-ulcerations-following-thymectomy
#8
Jung Min Lim, Sang Eun Lee, Jimyung Seo, Do Young Kim, Takashi Hashimoto, Soo-Chan Kim
Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Skin and mucosal biopsies identified interface dermatitis with lichenoid lymphocytic infiltration in the upper dermis...
April 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28390814/meeting-report-of-the-pathogenesis-of-pemphigus-and-pemphigoid-meeting-in-munich-september-2016
#9
Enno Schmidt, Volker Spindler, Rüdiger Eming, Masayuki Amagai, Frank Antonicelli, John F Baines, Meriem Belheouane, Philippe Bernard, Luca Borradori, Marzia Caproni, Giovanni Di Zenzo, Sergei Grando, Karen Harman, Marcel F Jonkman, Hiroshi Koga, Ralf J Ludwig, Andrew P Kowalczyk, Eliane J Müller, Wataru Nishie, Hendri Pas, Aimee S Payne, Christian D Sadik, Allan Seppänen, Jane Setterfield, Hiroshi Shimizu, Animesh A Sinha, Eli Sprecher, Michael Sticherling, Hideyuki Ujiie, Detlef Zillikens, Michael Hertl, Jens Waschke
Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the Departments of Dermatology in Lübeck and Marburg and the Institute of Anatomy and Cell Biology, Munich, was held in September 2016 in Munich. The meeting brought together basic scientists and clinicians from all continents dedicating their work to autoimmune blistering diseases...
April 5, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28378490/augenbeteiligung-beim-pemphigus-vulgaris-retrospektive-studie-an-einer-gro%C3%A3-en-spanischen-kohorte
#10
Agustin España, Pilar Iranzo, Josep Herrero-González, José M Mascaro, Ricardo Suárez
HINTERGRUND UND ZIELE: Es gibt nur wenige Berichte zur Beteiligung der okulären/periokulären Region beim Pemphigus vulgaris (PV). Ziel der vorliegenden Studie war es, das Krankheitsbild des okulären PV (OPV) zu untersuchen und seinen prognostischen Wert zu definieren. PATIENTEN UND METHODIK: Zwischen 1985 und 2014 wurden insgesamt 167 Patienten mit Pemphigus vulgaris an vier tertiären spanischen Krankenhäusern behandelt. Wir haben alle Patienten mit OPV in diese retrospektive Studie aufgenommen...
April 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/28378419/successful-treatment-of-pemphigus-foliaceus-in-a-berrichon-du-cher-ram-with-methylprednisolone-acetate
#11
Bianca Lambacher, Julia Schoiswohl, Rene Brunthaler, Thomas Wittek, Reinhild Krametter-Frötscher
BACKGROUND: Pemphigus foliaceus is a severe, autoimmune blistering skin disease, which is described in humans and some animal species. In small ruminants pemphigus foliaceus has rarely been described and, to the best of the authors' knowledge, little information is available about successful treatment in sheep. AIM: This case report describes a Berrichon du Cher ram with the presumed diagnosis of pemphigus foliaceus. METHODS: The ram was treated with methylprednisolone acetate 40 mg at a dosage of 2...
April 4, 2017: Veterinary Dermatology
https://www.readbyqxmd.com/read/28368874/efficacy-of-triaging-direct-immunofluorescence-in-intraepidermal-bullous-dermatoses
#12
Brian J Tjarks, Steven D Billings, Jennifer S Ko
BACKGROUND: Direct immunofluorescence (DIF) is considered pivotal in diagnosing autoimmune blistering diseases. Our goal was to examine the necessity of DIF in intraepidermal bullous cases, of which pemphigus vulgaris (PV) is the prototype. METHODS: Sixty-six cases from 2010 to 2014 submitted for DIF with an intraepidermal blistering disease listed in the differential diagnosis were reviewed by 2 board-certified dermatopathologists to see if they would order DIF based on routine histologic findings...
March 24, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28367488/atypical-laboratory-presentation-of-paraneoplastic-pemphigus-associated-with-castleman-disease
#13
Sarah Lonowski, Hayley Goldbach, Vanessa Holland
No abstract text is available yet for this article.
March 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28366912/low-dose-rituximab-as-an-adjuvant-therapy-in-pemphigus
#14
Jaya Gupta, Ranjan C Raval, Arti N Shah, Rekha B Solanki, Dhara D Patel, Kaksha B Shah, Ami D Badheka, Keyur B Shah, Neetish K Aggarwal, Vaaruni Ravishankar
BACKGROUND: Pemphigus is a chronic autoimmune blistering disease where systemic steroids and immunosuppressants are the mainstay of therapy, but long-term treatment with these agents is associated with many side effects. Rituximab, a chimeric monoclonal anti-CD20 antibody, in low doses has shown efficacy as an adjuvant to reduce the dose of steroids. AIM: To study the clinical efficacy and safety of low-dose rituximab as an adjuvant therapy in pemphigus. METHODS: Fifty patients with extensive pemphigus were selected, who either had recalcitrant pemphigus, were steroid dependent, had relapsed after pulse therapy, had anti-desmoglein levels >20, had contraindications to conventional treatment or wanted to avoid conventional treatment and its side effects...
March 29, 2017: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/28365191/-thymoma-and-autoimmune-diseases
#15
Y Jamilloux, H Frih, C Bernard, C Broussolle, P Petiot, N Girard, P Sève
The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Autoimmune diseases occur most often before thymectomy, but they can be discovered at the time of surgery or later...
March 29, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28361863/trichilemmal-cystis-in-metastatic-melanoma-a-case-report
#16
I Savarese, M Grazzini, A Gori, A D'Errico, L Doni, F Scarfì, P Covarelli, F Di Costanzo, V De Giorgi
The malignant melanoma is a neoplasm associated with a wide variety of cutaneous paraneoplastic syndromes, as dermatomyositis, systemic sclerosis, paraneoplastic pemphigus. We describe a case of four multiple trichilemmal cystis arising on frontal region in the same patient with brain metastasis and unknown primary melanoma and discuss their relationship.
March 2017: Experimental Oncology
https://www.readbyqxmd.com/read/28348966/cassia-fistula-a-remedy-from-traditional-persian-medicine-for-treatment-of-cutaneous-lesions-of-pemphigus-vulgaris
#17
REVIEW
Fatemeh Atarzadeh, Mohammad Kamalinejad, Ladan Dastgheib, Gholamreza Amin, Amir Mohammad Jaladat, Majid Nimrouzi
OBJECTIVE: Pemphigus is a rare autoimmune disease that may be fatal without proper medical intervention. It is a blistering disease that involves both the skin and mucus membranes, in which the most important causes of death comprise superimposed opportunistic infections and complications of long-term high-dose corticosteroid therapy or prolonged consumption of immune suppressant drugs. Skin lesions are the most important sources of infection, and any local treatment decreasing the healing time of lesions and reducing the total dosage of drugs is favorable...
March 2017: Avicenna Journal of Phytomedicine
https://www.readbyqxmd.com/read/28346642/nasal-oral-and-pharyngolaryngeal-manifestations-of-pemphigus-vulgaris-endoscopic-ororhinolaryngologic-examination
#18
Masafumi Ohki, Shigeru Kikuchi
Pemphigus vulgaris is an autoimmune blistering disorder that involves the skin and mucous membranes. Few reports have described nasal and oropharyngolaryngeal lesions in pemphigus vulgaris using an endoscopic ororhinolaryngologic examination. We retrospectively reviewed the clinical records of 11 patients with pemphigus vulgaris between 2001 and 2013 with respect to their symptoms, lesion sites, lesion features, and treatments received. All patients had undergone an endoscopic ororhinolaryngologic examination...
March 2017: Ear, Nose, & Throat Journal
https://www.readbyqxmd.com/read/28342637/first-line-rituximab-combined-with-short-term-prednisone-versus-prednisone-alone-for-the-treatment-of-pemphigus-ritux-3-a-prospective-multicentre-parallel-group-open-label-randomised-trial
#19
Pascal Joly, Maud Maho-Vaillant, Catherine Prost-Squarcioni, Vivien Hebert, Estelle Houivet, Sébastien Calbo, Frédérique Caillot, Marie Laure Golinski, Bruno Labeille, Catherine Picard-Dahan, Carle Paul, Marie-Aleth Richard, Jean David Bouaziz, Sophie Duvert-Lehembre, Philippe Bernard, Frederic Caux, Marina Alexandre, Saskia Ingen-Housz-Oro, Pierre Vabres, Emmanuel Delaporte, Gaelle Quereux, Alain Dupuy, Sebastien Debarbieux, Martine Avenel-Audran, Michel D'Incan, Christophe Bedane, Nathalie Bénéton, Denis Jullien, Nicolas Dupin, Laurent Misery, Laurent Machet, Marie Beylot-Barry, Olivier Dereure, Bruno Sassolas, Thomas Vermeulin, Jacques Benichou, Philippe Musette
BACKGROUND: High doses of corticosteroids are considered the standard treatment for pemphigus. Because long-term corticosteroid treatment can cause severe and even life-threatening side-effects in patients with this disease, we assessed whether first-line use of rituximab as adjuvant therapy could improve the proportion of patients achieving complete remission off-therapy, compared with corticosteroid treatment alone, while decreasing treatment side-effects of corticosteroids. METHODS: We did a prospective, multicentre, parallel-group, open-label, randomised trial in 25 dermatology hospital departments in France (Ritux 3)...
March 22, 2017: Lancet
https://www.readbyqxmd.com/read/28342636/rituximab-as-first-line-treatment-of-pemphigus
#20
Enno Schmidt
No abstract text is available yet for this article.
March 22, 2017: Lancet
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