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Mucous membrane pemphigoid

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https://www.readbyqxmd.com/read/28922328/minor-salivary-gland-transplantation-for-severe-dry-eyes
#1
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...
September 15, 2017: Cornea
https://www.readbyqxmd.com/read/28860044/clinical-implications-of-direct-immunofluorescence-dif-findings-in-patients-with-ocular-mucous-membrane-pemphigoid
#2
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 one year at the Duke University multidisciplinary ocular MMP clinic. Data was collected by chart review and included: patient demographics, clinical examination findings, history of autoimmune disease and/or malignancy, as well as topical, systemic, and surgical treatments received...
August 28, 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
#3
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...
August 30, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28801511/paraneoplastic-mucous-membrane-pemphigoid-with-ocular-and-laryngeal-involvement
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
https://www.readbyqxmd.com/read/28417469/oesophageal-involvement-in-26-consecutive-patients-with-mucous-membrane-pemphigoid
#16
O Zehou, J-J Raynaud, C Le Roux-Villet, M Alexandre, G Airinei, F Pascal, M Heller, N Lièvre, L Laroche, F Caux, R Benamouzig, C Prost-Squarcioni
BACKGROUND: Oesophageal involvement of mucous membrane pemphigoid (MMP) has not yet been thoroughly described. OBJECTIVES: This study was undertaken to systematically characterise the endoscopic lesions of a series of patients with oesophageal symptoms seen in a referral centre for autoimmune bullous diseases. METHODS: Clinical, endoscopic and immunological findings of consecutively referred MMP patients with oesophageal involvement, systemic and endoscopic treatments and follow-up, are described...
April 18, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28399039/characterization-of-corneal-involvement-in-eyes-with-mucous-membrane-pemphigoid-by-in-vivo-confocal-microscopy
#17
Tudor C Tepelus, Jianyan Huang, SriniVas R Sadda, Olivia L Lee
PURPOSE: To describe the morphological features of the corneal epithelial layers, subbasal nerve plexus, stroma, and endothelium in patients with mucous membrane pemphigoid (MMP) as shown by in vivo confocal microscopy (IVCM). METHODS: Central corneal images were captured from 10 healthy age-matched control eyes and 30 eyes with clinically diagnosed MMP by in vivo laser scanning confocal microscopy (HRT III RCM). Morphological changes of the corneal epithelial layers, stroma, and endothelium, characteristics of corneal nerves, and presence of inflammatory dendritic cells (DCs) were evaluated...
April 10, 2017: Cornea
https://www.readbyqxmd.com/read/28355312/oral-rehabilitation-of-a-severe-periodontally-involved-patient-with-mucous-membrane-pemphigoid-a-15-year-follow-up-case-report
#18
Jean-Marie Megarbane, Cécile Freiha, Nadim Mokbel
Mucous membrane pemphigoid (MMP) refers to a group of chronic autoimmune subepithelial diseases distinguished by erosive lesions of the mucous membranes and skin. Its treatment consists of inhibition of the inflammatory reaction by means of corticosteroids and symptomatic medication. This is a report of a patient suffering from a combination of MMP and severe generalized chronic periodontitis. The patient has been treated with oral corticosteroids, initial phase therapy, extraction with immediate implant placement, and periodontal surgery where the prognosis was questionable...
March 29, 2017: International Journal of Periodontics & Restorative Dentistry
https://www.readbyqxmd.com/read/28347855/role-of-high-resolution-optical-coherence-tomography-in-diagnosing-ocular-surface-squamous-neoplasia-with-coexisting-ocular-surface-diseases
#19
Marwan Atallah, Madhura Joag, Anat Galor, Guillermo Amescua, Afshan Nanji, Jianhua Wang, Victor L Perez, Sander Dubovy, Carol L Karp
PURPOSE: Coexistence of an ocular surface disease can mask the typical features of ocular surface squamous neoplasia (OSSN). The purpose of this study was to evaluate high resolution optical coherence tomography (HR-OCT) as an adjunct in the detection and differentiation of OSSN within coexisting ocular surface pathologies. METHODS: Retrospective study of 16 patients with ocular surface disease and lesions suspicious for OSSN that were evaluated with HR-OCT. HR-OCT images of the lesions were taken to look for evidence of OSSN...
March 24, 2017: Ocular Surface
https://www.readbyqxmd.com/read/28338219/human-orf-complicated-by-epidermolysis-bullosa-acquisita
#20
E Zuelgaray, C Salle de Chou, J Gottlieb, M Battistella, M D Vignon-Pennamen, M Bagot, F Guibal, J D Bouaziz
Orf is a DNA parapoxvirus transmitted to humans by contact with infected goats and sheep. Many complications have been reported after Orf infection including erythema multiforme. A few cases of auto-immune bullous dermatosis complicating Orf disease have been reported to date, usually characterized by tense blisters eruptions with or without mucosal involvement, linear deposition of C3, IgG and/or IgA along the basement membrane and negativity of indirect immunofluorescence analysis and ELISA assays (performed in 4 of 11 reported cases) against target antigens of bullous pemphigoid, mucous membrane pemphigoid or epidermolysis bullosa acquisita, except one case of mucosal pemphigoid with antilaminin-332 antibodies...
March 24, 2017: British Journal of Dermatology
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