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Mucous membrane pemphigoid

Geraint P Williams, Peter Nightingale, Sue Southworth, Alastair K O Denniston, Paul J Tomlins, Stephen Turner, John Hamburger, Simon J Bowman, S John Curnow, Saaeha Rauz
Purpose: Ocular mucous membrane pemphigoid (OcMMP) is a rare autoimmune disorder resulting in progressive conjunctival fibrosis and ocular surface failure leading to sight loss in up to 50%. This study was designed to optimize an ocular surface sampling technique for identification of novel biomarkers associated with disease activity and/or progressive fibrosis. Methods: Fifty-seven patients with OcMMP underwent detailed examination of conjunctival inflammation and fibrosis using fornix depth measurement...
October 1, 2016: Investigative Ophthalmology & Visual Science
Minhee Kim, Luca Borradori, Dédée F Murrell
Elderly patients are more susceptible to the development of autoimmune blistering disorders such as bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and paraneoplastic pemphigus. This article focuses on the clinical aspects of the aforementioned autoimmune blistering diseases and highlights the important factors involved in treating elderly patients. It is essential for clinicians to offer individualized treatment plans for these patients to optimize outcomes, as elderly patients often have multiple co-morbidities, polypharmacy, and suboptimal socioeconomic status that can adversely influence adequate compliance...
October 13, 2016: Drugs & Aging
Stefanie Haefliger, Michael P Horn, Valerie G A Suter, Michael M Bornstein, Luca Borradori
No abstract text is available yet for this article.
October 12, 2016: JAMA Dermatology
Michael Stormly Hansen, Oliver Niels Klefter, Hanne Olsen Julian, Anne Marie Lynge Pedersen, Steffen Heegaard
Pemphigoid and pemphigus diseases as well as Stevens-Johnson syndrome present as vesiculobullous disorders of the skin and may additionally involve both the oral cavity and the ocular surface. Ocular involvement ranges from mild irritation and dry eye disease to chronic conjunctivitis, symblepharon, eyelid malposition, ocular surface scarring and severe visual loss. In addition to diagnostic assessments, ophthalmologists must treat the dry eye and Meibomian gland dysfunction components of these diseases using a stepladder approach, including eyelid hygiene and lubricants...
October 7, 2016: Oral Diseases
Sarah D Ahadome, David J Abraham, Suryanarayana Rayapureddi, Valerie P Saw, Daniel R Saban, Virginia L Calder, Jill T Norman, Markella Ponticos, Julie T Daniels, John K Dart
Mucous membrane pemphigoid (MMP) is a systemic mucosal scarring disease, commonly causing blindness, for which there is no antifibrotic therapy. Aldehyde dehydrogenase family 1 (ALDH1) is upregulated in both ocular MMP (OMMP) conjunctiva and cultured fibroblasts. Application of the ALDH metabolite, retinoic acid (RA), to normal human conjunctival fibroblasts in vitro induced a diseased phenotype. Conversely, application of ALDH inhibitors, including disulfiram, to OMMP fibroblasts in vitro restored their functionality to that of normal controls...
August 4, 2016: JCI Insight
Bernhard Steger, Savitha Madhusudan, Stephen B Kaye, Amira Stylianides, Vito Romano, Sundas E Maqsood, Janice Harper, Sajjad Ahmad
PURPOSE: Despite the availability of systemic immunosuppressants, cicatricial conjunctivitis (CC) remains a potentially blinding ocular surface disease. We aim to describe the combined use of rituximab (RTX) and intravenous immunoglobulin (IVIg) for severe recalcitrant autoimmune CC. METHODS: In this single-center retrospective interventional case series with follow-up between 32 and 65 months, 3 cases with mucous membrane pemphigoid (patients 1-3) and 1 case with linear IgA disease (patient 4) were included...
September 21, 2016: Cornea
C Günther
Autoimmune bullous diseases are characterized by intraepidermal or subepidermal autoantibody deposition that leads to blisters and secondary erosion. Mucous membranes are frequently affected in pemphigus vulgaris and always involved in cicatricial and mucosal pemphigoid. Mucosal lesions are detected less frequently in patients with bullous pemphigoid or epidermolysis bullosa acquisita. The diagnosis of autoimmune bullous disorders is based on determination of the subtype of autoantibodies bound in the skin and the clinical picture...
October 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Julia Yu-Fong Chang, Chun-Pin Chiang, Yi-Ping Wang, Yang-Che Wu, Hsin-Ming Chen, Andy Sun
BACKGROUND: Desquamative gingivitis (DG) is principally associated with erosive oral lichen planus (EOLP), mucous membrane pemphigoid (MMP), and pemphigus vulgaris (PV). METHODS: Serum autoantibodies including antigastric parietal cell antibody (GPCA), antithyroglobulin antibody (TGA), and antithyroid microsomal antibody (TMA) were measured in 500 patients with DG, 287 EOLP without DG (EOLP/DG(-) ) patients, and 100 healthy control subjects. RESULTS: The 500 patients with DG were diagnosed as having EOLP in 455 (91%), PV in 40 (8%), and MMP in five (1%) patients...
September 7, 2016: Journal of Oral Pathology & Medicine
Veena Vijayan, Ajish Paul, Kennedy Babu, Balasubramanian Madhan
Desquamative gingivitis (DG) is a clinical condition in which the gingiva appears reddish, glazed, and friable with loss of superficial epithelium. DG is considered a clinical manifestation of many gingival diseases and hence not identified as a diagnosis itself. Mucous membrane pemphigoid (MMP) is an autoimmune vesiculobullous disorder of mucous membrane characterized by subepithelial bullae formation. MMP can affect the mucous membranes of oral cavity, conjunctiva, nasopharynx, larynx, esophagus, genitourinary tract, and anus and vary in its severity...
May 2016: Journal of Indian Society of Periodontology
Ramon Lee, Ziad Khoueir, Edem Tsikata, James Chodosh, Claes H Dohlman, Teresa C Chen
PURPOSE: To report the long-term visual outcomes and complications after Boston keratoprosthesis type II implantation in the largest single-center case series with the longest average follow-up. DESIGN: Retrospective review of consecutive clinical case series. PARTICIPANTS: Between January 1992 and April 2015 at the Massachusetts Eye and Ear Infirmary, 48 eyes of 44 patients had keratoprosthesis type II implanted by 2 surgeons (C.H.D. and J...
August 15, 2016: Ophthalmology
Alexandra Sperl, Johann W Bauer, Damian Meyersburg
The association of autoimmune bullous diseases (i.e., bullous pemphigoid, linear IgA disease, mucous membrane pemphigoid and IgA pemphigus) and inflammatory bowel disease, namely ulcerative colitis and Crohn's disease has formerly been reported. However, to our knowledge, we report herein the first case of lymphocytic colitis with concomitant bullous pemphigoid.
September 2016: Dermatology and Therapy
Paul A Gaudio, John Sinard, David R Shield, John J Huang, Brett A King
PURPOSE: To evaluate whether conjunctival biopsy findings in patients with ocular mucous membrane pemphigoid (MMP) persist as positive or revert to negative following treatment with immunomodulatory therapy (IMT). METHODS: Patients with biopsy-proven MMP were treated with IMT for at least 2 years before undergoing repeat conjunctival biopsy for immunofluorescence microscopy. Their records were reviewed and findings evaluated to ascertain which patients' biopsies showed antibody deposition on the conjunctival basement membrane...
July 20, 2016: Ocular Immunology and Inflammation
Clara C Chan, Lorena LoVerde, Judy Qiang, Michael L Nordlund, Edward J Holland
PURPOSE: To determine the rate of Boston type 1 keratoprosthesis (Kpro)-related corneal melts, leaks, and extrusions requiring surgical repair and to analyze possible risk factors and visual outcomes. METHODS: Retrospective chart review of 110 patients (128 eyes) who received a Kpro between November 2004 and December 2010 (average follow-up of 29 mo). The rate of corneal complications, risk factors for melts, and postmelt repair visual outcomes were evaluated. RESULTS: Twenty eyes from 18 patients developed Kpro-related melts requiring surgical repair...
August 2016: Cornea
Heng L Tham, Thierry Olivry, Keith E Linder, Petra Bizikova
BACKGROUND: Mucous membrane pemphigoid (MMP) is a chronic autoimmune subepidermal blistering disease of dogs, cats and humans. OBJECTIVES: The goal of this study was to describe the clinical, histological and immunological features and treatment outcomes of canine MMP. ANIMALS: Sixteen dogs were diagnosed with MMP based on the presence of mucosal- or mucocutaneous-predominant vesiculation and/or ulceration, histological confirmation of subepidermal clefting and an age of disease onset greater than 6 months...
October 2016: Veterinary Dermatology
E Schmidt
No abstract text is available yet for this article.
May 2016: British Journal of Dermatology
Xiaoguang Li, Hua Qian, Ryosuke Sogame, Yoshiaki Hirako, Daisuke Tsuruta, Norito Ishii, Hiroshi Koga, Atsunari Tsuchisaka, Zhexiong Jin, Kazuo Tsubota, Akiko Fukumoto, Chie Sotozono, Shigeru Kinoshita, Takashi Hashimoto
Previous studies of ocular mucous membrane pemphigoid (OMMP) have identified several components of the basement membrane zone to be autoantigens, including integrin β4. However, there are no extensive or definitive reported studies that address this, particularly in pure OMMP. To clarify the major autoantigens in pure OMMP. In this study, we examined sera from 43 pure OMMP patients for both IgG and IgA antibodies using newly developed immunoblotting analyses with a hemidesmosome-rich fraction and various recombinant proteins of integrin α6β4, in addition to our routine immune-serological tests...
June 1, 2016: European Journal of Dermatology: EJD
Robert Nash, Jonathon Hughes, Romana Kuchai, Ann Sandison, Guri Sandhu
Mucous membrane pemphigoid (MMP) comprises a heterogenous spectrum of rare subepithelial blistering disorders affecting characteristic tissues throughout the body(1) . The prevalence is approximately 5/10,000(2) . Scarring is the clinical hallmark, but may not always be obvious(3) . MMP may affect mucosae, the cornea and the skin; and commonly affected sites include the oral cavity, eye, sinonasal cavity, and the genitals. This article is protected by copyright. All rights reserved.
May 14, 2016: Clinical Otolaryngology
Joost M Meijer, Gilles F H Diercks, Enno Schmidt, Hendri H Pas, Marcel F Jonkman
IMPORTANCE: Anti-p200 pemphigoid is a rare subepidermal autoimmune blistering disease characterized by autoantibodies against a 200-kDa protein in the basement membrane zone. Anti-p200 pemphigoid is probably often misdiagnosed because of low availability of diagnostic assays and expertise and classified as bullous pemphigoid or epidermolysis bullosa acquisita. OBJECTIVE: To clinically characterize patients with anti-p200 pemphigoid, identified by using indirect immunofluorescence microscopy on skin substrates deficient in type VII collagen and laminin-332 (knockout analysis), to validate this technique by immunoblot with dermal extract, and to incorporate direct immunofluorescence serration pattern analysis in the diagnostic algorithm...
August 1, 2016: JAMA Dermatology
E Szabó, M Palos, P Skalická
INTRODUCTION: Ocular cicatricial pemphigoid (OCP) is rare, severe, sight threatening autoimmune disease of the conjunctiva, which affects elderly patients, more often women. AIM: To evaluate the success rate of stabilisation of ocular findings in patients with OCP. METHODS: Retrospective study of patients from Centre of Conjunctival and Corneal Diseases at Department of Ophthalmology, General University Hospital and 1st Medical Faculty of Charles University in Prague in 1992-2013 was performed...
February 2016: Ceská a Slovenská Oftalmologie
Mihai Radulescu
The oral ulcerations caused by aphtous lesions, herpetic lesions, candidiasis, ulcerative lichen planus, mucous membrane pemphigoid, and pemphigus vulgaris are managed in a step-up approach that can involve topical, intarlesional, and systemic pharmacologic management. This article reviews the common treatment agents, modalities, and dosages. The emphasis is on local pharmacologic therapies, yet systemic conditions that often present with such oral lesions are briefly reviewed, along with the appropriate management...
April 2016: Dental Clinics of North America
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