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Mucous membrane pemphigoid

Emanuele Cozzani, Giovanni Di Zenzo, Margherita Cioni, Sanja Javor, Marco Altieri, Luca Anselmi, Aurora Parodi
No abstract text is available yet for this article.
March 16, 2018: European Journal of Dermatology: EJD
Aniek Lamberts, H Ilona Euverman, Jorrit B Terra, Marcel F Jonkman, Barbara Horváth
Introduction: Rituximab (RTX) is a monoclonal antibody targeting CD20, a transmembrane protein expressed on B cells, causing B cell depletion. RTX has shown great efficacy in studies of pemphigus vulgaris, but data of pemphigoid diseases are limited. Objective: To assess the effectiveness and safety of RTX in pemphigoid diseases. Methods: The medical records of 28 patients with pemphigoid diseases that were treated with RTX were reviewed retrospectively...
2018: Frontiers in Immunology
Matthew Howard, Anthony Hall
Vulval lichen planus-lichen sclerosus overlap is an emerging observation. Few clinical reports exist with no reviews of literature. We present a focused update of this phenomenon and discuss a clinical case. We report a 63-year-old woman with a 20-year history of ulcerative vulvo-vaginitis, initially diagnosed as benign mucous membrane (cicatricial) pemphigoid. This led to prolonged treatment with oral corticosteroids with minimal improvement in symptoms. Subsequent complications of long-term use of systemic corticosteroid ensued...
January 1, 2018: International Journal of STD & AIDS
J Bagan, Y Jiménez, J Murillo, L Bagan
OBJECTIVE: To analyze the severity of the oral lesions in low-risk oral mucous membrane pemphigoid (OMMP) measured according to the size of the bullous areas and the number of simultaneously affected oral locations. MATERIAL AND METHODS: A total of 100 cases of low-risk OMMP were studied. The symptoms and location of OMMP in the oral cavity were analyzed. The bullous areas were measured, establishing three grades according to the greatest bullous lesion size (grade 1: < 3 cm in size; grade 2: 3-6 cm; and grade 3: > 6 cm)...
March 2018: Oral Diseases
Giorgia Capocasale, Vera Panzarella, Vito Rodolico, Olga Di Fede, Giuseppina Campisi
There is currently a growing interest in new diagnostic tools of the oral cavity and mucosa which are non-invasive, repeatable and reliable. A diagnosis of a suspected, autoimmune pathology was made regarding a 57-year-old patient with desquamative gingivitis. However, a negative Nikolsky's sign did not seem to indicate a diagnosis of mucous membrane pemphigoid neither was there any indication as to the optimum location for an incisional biopsy. As an imaging method, the use of optical coherence tomography (OCT) has enabled the obtaining of tomographic (cross-sectional) scans of tissue...
February 26, 2018: Journal of Dermatology
Mayumi Kamaguchi, Hiroaki Iwata, Inkin Ujiie, Hideyuki Ujiie, Jun Sato, Yoshimasa Kitagawa, Hiroshi Shimizu
Mucous membrane pemphigoid (MMP) is a rare organ-specific autoimmune subepithelial blistering disease with predominantly mucosal erosions, most frequently affecting the gingiva. Erosions in the oral cavity usually result in markedly decreased quality of life. The major autoantigens are BP180 and laminin332, which are components of basement membrane proteins in the skin and mucosa. Diagnosis is usually difficult due to histological destruction of the tissue and low autoantibody titers. In this study, we evaluated the diagnostic value of direct immunofluorescence (DIF) using non-lesional buccal mucosa in seven cases of MMP...
2018: Frontiers in Medicine
Mareike Witte, Detlef Zillikens, Iakov Shimanovich
Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease that is notoriously difficult to treat. Rituximab (anti-CD20) and high-dose intravenous immunoglobulins (IVIG) have both been reported to be effective in refractory MMP.1,2Furthermore, treatment regimens based on the concomitant use of rituximab and IVIG in ocular MMP have been published.3,4In the current report, we present 5 patients with treatment-resistant MMP who failed to respond to rituximab but rapidly achieved remission on IVIG. This article is protected by copyright...
February 14, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Carolyn J Kushner, Josef Symon S Concha, Victoria P Werth
Autoimmune bullous diseases (AIBD), including pemphigus, bullous pemphigoid, epidermolysis bullosa acquisita, mucous membrane pemphigoid, and pemphigoid gestationis, pose significant therapeutic challenges, especially in pregnant and post-partum breastfeeding patients or those planning to conceive. Data on the safety and efficacy of therapeutic interventions during the perinatal period are lacking because randomized controlled trials are typically not performed in this setting. However, many of the treatments for AIBD are also used in other diseases, so data can be extrapolated from studies or case reports in these other patient populations...
February 2, 2018: American Journal of Clinical Dermatology
Lina Saeed, Timothy H Schmidt, Lianne S Gensler, Andrew J Gross, Lindy P Fox, Tiffany C Scharschmidt, Karin Gaensler, Haley Naik, Michael A Rosenblum, Kanade Shinkai
No abstract text is available yet for this article.
January 2018: JAAD Case Reports
Jayesh Vazirani, Dhanyasree Nair, Swapna Shanbhag, Siva Wurity, Abhishek Ranjan, Virender Sangwan
PURPOSE: To determine the demographic features of patients affected by limbal stem cell deficiency (LSCD), and to identify the underlying causes of LSCD DESIGN: Retrospective, multi-center case series SETTING: Two large tertiary care ophthalmology hospitals SUBJECTS: Patients with a diagnosis of LSCD presenting from January 1, 2005 to December 31, 2014 METHODS: Records of patients with a clinical diagnosis of LSCD were reviewed. Demographic details and clinical features at presentation, as well as the underlying cause of LSCD (if identified) were noted...
January 26, 2018: American Journal of Ophthalmology
Thomas Schmidt, Susanne Mauracher, Lena Bender, Brandon Greene, Jonas Kurzhals, Rüdiger Eming, Ralf Dostatni, Michael Hertl
Pemphigus vulgaris (PV) is a severe autoimmune blistering disease of the skin and mucous membranes. As autoantibodies play an essential role in the disease pathogenesis, the serological detection of anti-desmoglein 3 IgG represents a central tool in the diagnosis of the disease. In this study, we show the validation of a novel lateral flow immunoassay (LFIA) which rapidly detects anti-desmoglein 3 (Dsg3) IgG in human serum. In contrast to other diagnostic procedures, the assay is compact and simple to perform and delivers a fast "yes" or "no" answer within 10 min without additional hardware requirements for test evaluation...
December 26, 2017: Experimental Dermatology
M M Holtsche, D Zillikens, E Schmidt
Mucous membrane pemphigoid (MMP) is a pemphigoid disease defined by the presence of autoantibodies against the dermal-epidermal junction and predominant involvement of mucous membranes. Diagnosis is made by the clinical presentation and linear deposits of IgG and/or IgA and/or C3 at the dermal-epidermal junction by direct immunofluorescence microscopy of a perilesional biopsy. Circulating autoantibodies can be detected in most patients by indirect immunofluorescence microscopy on salt-split human skin as well as ELISA and immunoblotting with recombinant and cell-derived target antigens...
January 2018: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
J Loyal, S Rashtak
Lichen planus pemphigoides (LPP) is a rare blistering disease with features of both lichen planus and bullous pemphigoid. LPP typically appears on the extremities and occasionally involves the oral mucosa. Herein, we describe a case of LPP of the vulva of an 80-year-old woman, an uncommon location for this disease process. This clinical scenario can be confused with a number of similarly appearing entities such as erosive vulvar lichen planus, mucous membrane pemphigoid, and erosive lichen sclerosus et atrophicus...
December 2017: International Journal of Women's Dermatology
Hon Shing Ong, Jane F Setterfield, Darwin C Minassian, John K Dart
PURPOSE: This study explored the validity of the First International Consensus on Mucous Membrane Pemphigoid (MMP) guidance, which recommends that clinically indistinguishable patients, who have direct immunofluorescence (DIF)-negative biopsies, be excluded from a diagnosis of MMP. Misdiagnosis, or delayed diagnosis, of MMP with ocular involvement leads to the inappropriate use of topical therapy, the standard of care for causes of cicatrising conjunctivitis other than MMP, rather than systemic immunomodulatory therapy, resulting in irreversible clinical deterioration in patients with MMP...
December 4, 2017: Ophthalmology
Emi Nishida, Eiichi Nishio, Hiroko Murashima, Norito Ishii, Takashi Hashimoto, Akimichi Morita
Subepidermal autoimmune blistering disease including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, anti-laminin-γ1 pemphigoid, linear immunoglobulin A bullous disease and epidermolysis bullosa acquisita (EBA), are all characterized by direct immunofluorescence microscopy or immunoglobulin deposition on the basement membrane zone. Among them, EBA is a rare acquired subepidermal autoimmune blistering disease of the skin and mucous membranes reactive with type VII collagen, a major component of the epidermal basement membrane zone...
December 4, 2017: Journal of Dermatology
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
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
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
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
H Yuan, M Pan
No abstract text is available yet for this article.
October 2017: British Journal of Dermatology
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