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Mucous membrane pemphigoid

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https://www.readbyqxmd.com/read/29159697/subepidermal-autoimmune-bullous-diseases-overview-epidemiology-and-associations
#1
REVIEW
Khalaf Kridin
Subepidermal autoimmune bullous diseases of the skin and mucosae comprise a large group of chronic diseases, including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. These diseases are characterized by an antibody response toward structural components of the basement membrane zone, resulting in subepidermal blistering. The epidemiological features of these diseases vary substantially in different regions of the world...
November 21, 2017: Immunologic Research
https://www.readbyqxmd.com/read/29136295/genome-wide-association-study-identifies-galc-as-susceptibility-gene-for-mucous-membrane-pemphigoid
#2
C D Sadik, J Bischof, N van Beek, A Dieterich, S Benoit, M Sárdy, M Worm, S Meller, R Gläser, D Zillikens, B Homey, J Setterfield, D Minassian, E Schmidt, J Dart, S M Ibrahim
BACKGROUND: Mucous membrane pemphigoid (MMP) is a rare, chronic, and often aggressive subepidermal autoimmune blistering disease potentially affecting several mucous membranes with blisters and secondary erosions and scars. The pathogenesis of MMP is poorly understood, and the contribution of genetic predispositions, other than HLA class II allele variants to MMP, is unknown. OBJECTIVES: The objective of this study is to identify susceptibility genes for MMP in a British cohort of MMP patients...
November 14, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/29131038/bullous-diseases
#3
Caroline Corbaux, Pascal Joly
Autoimmune bullous diseases are a heterogeneous group of blistering diseases affecting the skin and/or mucous membrane. Systemic corticosteroids, which are often associated with immunosuppressants, are the main treatment option for these diseases. The 2 main biologics used in the treatment of autoimmune bullous diseases are rituximab, especially in pemphigus and mucous membrane pemphigoid, and omalizumab in bullous pemphigoid. Rituximab is a promising therapeutic option in pemphigus and mucous membrane pemphigoid...
2018: Current Problems in Dermatology
https://www.readbyqxmd.com/read/29130487/a-case-of-mucous-membrane-pemphigoid-with-igg-antibodies-against-the-%C3%AE-3-and-%C3%AE-2-subunits-of-laminin-332-and-the-c-terminal-domain-of-bp180
#4
Makiko Yasumizu, Hisayoshi Imanishi, Shosuke Morita, Aoi Fukuda, Chiharu Tateishi, Hiroshi Koga, Takashi Hashimoto, Daisuke Tsuruta
No abstract text is available yet for this article.
November 11, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/29052880/endoscopic-characteristics-of-oesophagus-involvement-in-mucous-membrane-pemphigoid
#5
COMMENT
H Yuan, M Pan
No abstract text is available yet for this article.
October 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28922328/minor-salivary-gland-transplantation-for-severe-dry-eyes
#6
Tais Hitomi Wakamatsu, Ana Estela Besteti Pires Ponce SantʼAnna, Priscila Cardoso Cristovam, Venâncio Avancini Ferreira Alves, Alda Wakamatsu, José Alvaro Pereira Gomes
Dry eye is a multifactorial disease comprising a wide spectrum of ocular surface alterations and symptoms of discomfort. In most patients with aqueous-deficient dry eye, pharmaceutical tear substitutes are used to control symptoms and prevent ocular surface damage. However, in severe dry eye conditions caused by cicatricial disorders, such as Stevens-Johnson syndrome and ocular cicatricial mucous membrane pemphigoid, noninvasive treatments are insufficient, and patients are at risk of developing complications that can lead to blindness...
November 2017: Cornea
https://www.readbyqxmd.com/read/28860044/clinical-implications-of-direct-immunofluorescence-findings-in-patients-with-ocular-mucous-membrane-pemphigoid
#7
Mary T Labowsky, Sandra S Stinnett, Jason Liss, Melissa Daluvoy, Russell P Hall, Christine Shieh
PURPOSE: To examine the clinical implications of positive or negative direct immunofluorescence biopsies (DIF) in patients with clinically typical ocular mucous membrane pemphigoid (MMP). DESIGN: Retrospective cohort study. METHODS: The study population was patients with clinically typical ocular MMP disease with documented DIF results who were followed for at least 1 year at the Duke University multidisciplinary ocular MMP clinic. Data were collected by chart review and included patient demographics, clinical examination findings, and history of autoimmune disease and/or malignancy, as well as topical, systemic, and surgical treatments received...
November 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/28854309/determining-the-incidence-of-pneumocystis-pneumonia-in-patients-with-autoimmune-blistering-diseases-not-receiving-routine-prophylaxis
#8
Kyle T Amber, Aniek Lamberts, Farzan Solimani, Arianna F Agnoletti, Dario Didona, Ilona Euverman, Emanuele Cozzani, Lee Haur Yueh, Giovanni Di Zenzo, Yael Anne Leshem, Daniel Mimouni, Michael Hertl, Barbara Horvath
Importance: Pneumocystis pneumonia (PCP) is a potentially lethal opportunistic infection that primary prophylaxis can help prevent. The risk of prophylactic therapy must be weighed against the incidence of PCP in the patient population. Prophylaxis most frequently involves trimethoprim-sulfamethoxazole, with second-line therapies, including atovaquone, dapsone, and pentamide. The indication for prophylaxis in immunocompromised patients without HIV is less well defined. Previously, an incidence of at least 3...
November 1, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28801511/paraneoplastic-mucous-membrane-pemphigoid-with-ocular-and-laryngeal-involvement
#9
Silvia Lambiel, Pavel Dulguerov, Emmanuel Laffitte, Igor Leuchter
A 73-year-old woman was treated 8 years previously for synchronous breast and uterine neoplasms. She presented with a severe sore throat, odynophagia, dysphonia, dyspnoea, ocular irritation and weight loss over the last 3 months. Physical examination revealed ulcerations in the oral cavity, posterior pharyngeal wall and supraglottic larynx, nasal crusting, bilateral conjunctivitis and three cutaneous blisters. A diagnosis of anti-laminin 5 mucous membrane pemphigoid was retained, based on skin biopsy, direct immunofluorescence and immunoprecipitation...
August 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28764581/skin-diseases-of-the-vulva-inflammatory-erosive-ulcerating-and-apocrine-gland-diseases-zinc-and-vitamin-deficiency-vulvodynia-and-vestibulodynia
#10
Freja Lærke Sand, Simon Francis Thomsen
Chronic, inflammatory and ulcerating mucocutaneous diseases that can affect the vulvar area are reviewed: lichen sclerosus, lichen planus, plasma cell vulvitis, complex aphthosis, Behcet's disease, pyoderma gangrenosum, metastatic Crohn's disease, dyskeratotic skin diseases (Hailey-Hailey disease and Darier's disease), autoimmune bullous diseases (mucous membrane pemphigoid and pemphigus vulgaris) and hidradenitis suppurativa. Also, vulvodynia and vestibulodynia, zinc and vitamin B deficiency are described.
August 1, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28761292/analysis-of-serum-cytokine-profile-in-pemphigus
#11
Sang Hee Lee, Won Jin Hong, Soo-Chan Kim
BACKGROUND: Pemphigus is a group of autoimmune blistering diseases affecting skin and mucous membranes. While pemphigus is an autoantibody mediated disease, the role of T cells and cytokines in the pathogenesis is being increasingly recognized. OBJECTIVE: This study was conducted to observe alterations in the serum cytokine levels of patients with pemphigus vulgaris (PV), pemphigus foliaceous (PF), paraneoplastic pemphigus (PNP) and compare with bullous pemphigoid (BP) and healthy subjects...
August 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28742296/mucocutaneous-diseases-oral-lichen-planus-mucous-membrane-pemphigoid-and-pemphigus-vulgaris
#12
Seena Patel, Satish Kumar, Joel M Laudenbach, Antonia Teruel
Mucocutaneous diseases affect the oral cavity and can present a diagnostic challenge. They can have systemic involvement, necessitating multidisciplinary management. Frequently, patients will see their general dentists initially for evaluation. A better understanding of mucocutaneous diseases can prevent delay in appropriate diagnosis and treatment. Oral lichen planus, mucous membrane pemphigoid and pemphigus vulgaris are three mucocutaneous diseases that affect the oral mucosa. This review describes the clinical features, epidemiology, etiology, pathogenesis and management for each condition...
September 2016: Journal—California Dental Association
https://www.readbyqxmd.com/read/28705276/evaluation-of-autoimmune-bullous-diseases-in-elderly-patients-in-iran-a-10-year-retrospective-study
#13
Maryam Ghiasi, Maryam Daneshpazhooh, Muhammadkhuja Ismonov, Cheyda Chams-Davatchi
Autoimmune bullous diseases (ABDs) are uncommon but significant skin disorders with relatively high morbidity and mortality. Some surveys have been carried out to describe the spectrum of ABDs in a region, but this is the first that has focused on ABDs in elderly patients. This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Medical records of all ABD patients with disease onset after the age of 60 years who presented to the Autoimmune Bullous Diseases Research Center, Tehran, Iran between April 2003 and March 2013 were reviewed...
2017: Skinmed
https://www.readbyqxmd.com/read/28676329/multiple-and-repeated-sampling-increases-the-sensitivity-of-direct-immunofluorescence-testing-for-the-diagnosis-of-mucous-membrane-pemphigoid
#14
Iakov Shimanovich, Julia Marie Nitz, Detlef Zillikens
BACKGROUND: Mucous membrane pemphigoid (MMP) is an autoimmune disease characterized by the predominant blistering of mucosal surfaces and the linear deposition of complement, IgG, or IgA along the basement membrane detected by direct immunofluorescence (DIF) test. OBJECTIVE: To assess the impact of multiple and repeated DIF sampling on establishing the diagnosis of MMP. METHODS: We reviewed the results of DIF studies in 136 nonlesional biopsies from 78 patients who were immunologically confirmed to have MMP...
October 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28670088/linear-iga-bullous-dermatosis-mimicking-oral-lichen-planus
#15
Sheevam Shah, Brooke Mohr, Palak Parekh
Linear IgA bullous dermatosis (LABD) is an autoimmune disease characterized by blisters on the skin and mucous membranes. Polycyclic, grouped bullae on cutaneous skin are the most characteristic clinical feature of LABD. Lesions are often indistinguishable from bullous pemphigoid or may resemble dermatitis herpetiformis. Oral lesions may be seen in 5% to 70% of patients with LABD, and in some cases, the oral mucosa may be the only area of involvement. Herein, we report a patient with LABD involving only the oral mucosa clinically...
July 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28634600/oral-mucous-membrane-pemphigoid-associated-with-hypothyroidism-a-retrospective-study-and-a-case-report
#16
Ann Siassipour, Joseph Katz
OBJECTIVE: The purpose of the study was to evaluate the relationship between mucous membrane pemphigoid and a state of hypothyroidism. The study included analysis of the dental charts of 40 subjects with clinically and histologically confirmed diagnosis of mucous membrane pemphigoid and 35 age- and gender-matched subjects, from the same clinic, with no diagnosis of vesiculo-ulcerative lesions. RESULTS: Thirteen subjects from the pemphigoid group (32%) had a hypothyroid condition, compared to four subjects (11%) from the control group (P = ...
2017: Quintessence International
https://www.readbyqxmd.com/read/28533859/-a-particular-type-of-cicatricial-pemphigoid-with-unique-iga-deposit
#17
Amina Aounallah, Mariem Jrad, Mehdi Ksiaa, Sana Mokni, Wafa Saidi, Lobna Boussofara, Badreddine Sriha, Mohamed Denguezli, Najet Ghariani, Colandane Belajouza, Rafia Nouira
Cicatricial Pemphigoid is a subepithelial bullous dermatosis which essentially involves the mucous membranes with cicatricial evolution We report the case of a 66-year old patient hospitalized with erosive gingivitis associated with dysphagia, dyspnea and blurred vision. Dermatologic examination showed erosive lesions involving the palate and the pharynx. Ophthalmologic examination showed symblepharons, ectropion and bilateral cataract. Gingival biopsy revealed a necrotic detachment of the buccal epithelium...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28513499/role-of-mini-scleral-lens-in-mucous-membrane-pemphigoid
#18
Mukesh Kumar, Rohit Shetty, Chaitra Jayadev
This study aims to report the use of mini-scleral contact lens in the management of mucous membrane pemphigoid (MMP) with persistent epithelial defects. A 68-year-old male with a history of ocular pain and declining visual acuity was referred to our clinic with a diagnosis of MMP. His corrected distance visual acuity (CDVA) was 20/200 in the right eye and 20/100 in the left eye. Despite being on lubricants, topical steroids and intravenous cyclophosphamide, the patient developed persistent corneal epithelial defects in both eyes...
April 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28479314/epiglottitis-as-the-presenting-sign-of-mucous-membranous-pemphigoid-a-case-report
#19
Narin Nard Carmel-Neiderman, Ilana Kaplan, Yael Oestreicher-Kedem
Mucous membranous pemphigoid (MMP) is an autoimmune disease of the mucous membranes characterized by formation of subepithelial blisters. MMP commonly involves the ocular and oral mucosa. Laryngeal MMP is very rare, estimated as occurring in 1 of 10 million persons in the general population. It mostly affects the supraglottis, but may also involve other laryngeal sites as well as extralaryngeal areas. This report describes the clinical picture, workup to diagnosis, and differential diagnosis of an isolated epiglottic process in an elderly female who presented with isolated, long-standing, nonresolving epiglottitis, later diagnosed as MMP with epiglottal and oral involvement...
May 4, 2017: Journal of Voice: Official Journal of the Voice Foundation
https://www.readbyqxmd.com/read/28456612/experimental-laminin-332-mucous-membrane-pemphigoid-critically-involves-c5ar1-and-reflects-clinical-and-immunopathological-characteristics-of%C3%A2-the%C3%A2-human-disease
#20
Eva Nina Heppe, Sabrina Tofern, Franziska S Schulze, Akira Ishiko, Atsushi Shimizu, Christian Sina, Detlef Zillikens, Jörg Köhl, Stephanie Goletz, Enno Schmidt
Mucous membrane pemphigoid is an autoantibody-mediated disease predominantly affecting the oral cavity, pharynx, and conjunctiva. Conjunctival lesions may lead to impaired vision and, finally, blindness. About 25% of mucous membrane pemphigoid patients generate autoantibodies against the α3 chain of laminin 332 (LAMα3), a structural protein of epidermal/epithelial basement membranes. Here, we established a mouse model by the passive transfer of rabbit IgG against the murine homologs of two immunodominant fragments in adult C57BL/6 mice (mLAMα3)...
August 2017: Journal of Investigative Dermatology
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