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Jennifer E Thorne - One of the best experts on this subject based on the ideXlab platform.
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disease relapse after drug free remission in ocular Mucous Membrane Pemphigoid
American Journal of Ophthalmology, 2021Co-Authors: Amde Selassie Shifera, Jennifer E Thorne, Gloria H Hong, Chinwenwa Okeagu, Irfan KhanAbstract:Purpose To quantitate the risk of relapse of ocular and extraocular disease among patients with Mucous Membrane Pemphigoid (MMP) who had undergone drug-free remission. Design Retrospective, comparative, interventional case series. Methods There were 167 patients with biopsy-proven MMP who were seen at the Wilmer Eye Institute between November 1984 and December 2019. Among the 167 patients, 119 patients had ocular involvement and 103 of those patients received systemic treatment for MMP. The main outcome measures were the incidence of ocular remission, incidence rate of disease relapse after remission, and risk factors for disease relapse. Results Over a median follow-up time of 7 years, 74 of 103 treated patients (71.8%) experienced drug-free remission (incidence rate = 0.28/person-year [PY], 95% confidence interval [CI] 0.22–0.35/PY). Most patients (80/103, 77.7%) received cyclophosphamide therapy. Thirteen of the 74 patients (17.6%) had disease relapse after remission: 4 with ocular disease only, 4 with extraocular disease only, and 5 with both. The rate relapse of ocular MMP was 0.020/PY (95% CI 0.009–0.038/PY), and the rate of relapse of MMP at any site (ocular or extraocular site) was 0.029/PY (95% CI 0.015–0.050/PY). The use of cyclophosphamide was associated with a greater chance of remission (hazard ratio [HR] = 3.84, P Conclusions Rates of relapse of MMP after drug-free remission are low but not zero; therefore, monitoring of patients remains necessary. Relapses were not observed among those patients treated with rituximab who had remission; however, follow-up duration in those patients was shorter than the whole MMP cohort and the sample size was small.
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incidence and clinical characteristics of ocular involvement in Mucous Membrane Pemphigoid
Ocular Immunology and Inflammation, 2019Co-Authors: Gloria H Hong, Amde Selassie Shifera, Irfa R Kha, Chinwenwa Okeagu, Jennifer E ThorneAbstract:Purpose: To describe the risk of developing ocular Mucous Membrane Pemphigoid (MMP) or a new extraocular site of MMP, and to identify risk factors for new involvement. Methods: Retrospective chart review of 162 biopsy-proven MMP patients. Results: At presentation, 109 of 162 MMP patients (67.3%) had ocular involvement and 53 patients did not. Of the 53 patients without ocular involvement at presentation followed up to 22 years, the risk of developing ocular MMP was 0.014 per person-year (PY, 95% confidence interval [CI]: 0.005/PY, 0.034/PY). The risk of developing any new location of extraocular MMP was 0.020/PY (95% CI: 0.007/PY, 0.043/PY). Smoking was a risk factor for developing an additional extraocular MMP location (hazard ratio [HR] = 4.09, p = 0.04). Conclusions: Patients presenting with extraocular MMP are at risk for developing ocular MMP, and all MMP patients are at risk for developing secondary extraocular MMP locations, although the rates were low.
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Mucous Membrane Pemphigoid and pseudoPemphigoid
Ophthalmology, 2004Co-Authors: Jennifer E Thorne, Douglas A Jabs, Grant James AnhalAbstract:Abstract Purpose To describe the clinical characteristics of patients with Mucous Membrane Pemphigoid (MMP) and pseudoPemphigoid. Design Retrospective cohort study. Participants Two hundred eighty consecutive patients referred for the evaluation of possible ocular MMP from January 1, 1985, to December 31, 2001. Methods Information on patients presenting for evaluation of possible MMP was entered prospectively into a database, which was supplemented by a retrospective chart review. Mucous Membrane Pemphigoid was diagnosed in patients with a compatible clinical picture by the linear deposition of antibodies to the basement Membrane zone (BMZ) on direct immunofluorescent analysis of a Mucous Membrane biopsy specimen or by the presence of circulating autoantibodies to epithelial BMZ. Main outcome measures Demographic and clinical characteristics of MMP and pseudoPemphigoid; risk of ocular MMP among patients presenting with extraocular MMP without ocular disease. Results Among patients with ocular MMP, extraocular disease was common (82.4% of patients). The risk of ocular involvement among patients with MMP seen without ocular disease was approximately 5% per year over the first 5 years of follow-up (cumulative risk at 5 years, 22%). Although immunohistologic confirmation of the diagnosis was obtained in all patients, the initial conjunctival biopsy was positive for MMP in 80% of the patients diagnosed with ocular MMP. The most frequent presumed causes of pseudoPemphigoid were topical glaucoma medications (28.3%), rosacea blepharoconjunctivitis (20.0%), atopic keratoconjunctivitis (8.3%), and conjunctival lichen planus (8.3%). Conclusions Patients with ocular MMP typically have other systemic manifestations of MMP. Patients who are initially seen with extraocular MMP without ocular involvement are at risk for ocular disease developing. The clinical characteristics of ocular MMP and pseudoPemphigoid are similar; therefore, immunohistologic evaluation of biopsied tissue is needed to confirm the diagnosis of MMP.
Daisuke Tsuruta - One of the best experts on this subject based on the ideXlab platform.
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four cases of Mucous Membrane Pemphigoid with clinical features of oral lichen planus
International Journal of Dermatology, 2016Co-Authors: Aoi Fukuda, Hiroshi Koga, Daisuke Tsuruta, Akio Himejima, Ungo Ohyama, Shosuke MoritaAbstract:BACKGROUND Mucous Membrane Pemphigoid (MMP) and oral lichen planus (OLP) show similar clinical features on the oral mucosa. As clinical manifestations of oral mucosal lesions, MMP shows blisters and erosions, whereas OLP shows lace-like whitish lesions in an annular arrangement with erythema and erosions. Histopathologically, MMP shows subepithelial bullae with infiltrates of lymphocytes and neutrophils, whereas OLP shows band-like interface infiltration of lymphocytes with damage in basal cells. However, these two diseases are frequently difficult to distinguish both clinically and histopathologically. OBJECTIVES We report four patients with oral MMP who showed OLP-like clinical and histopathological lesions. METHODS We performed direct immunofluorescence, indirect immunofluorescence of normal human skin and 1 m NaCl-split skin, enzyme-linked immunosorbent assays for BP180, BP230, and desmogleins 1 and 3, and immunoblotting of normal human epidermal and dermal extracts, recombinant proteins of BP180-NC16a and -C-terminal domains, concentrated culture supernatant of HaCaT cells, and purified laminin-332. RESULTS The results of various immunological studies suggested the diagnoses of various types of MMP for all four patients. CONCLUSIONS Because MMP and OLP require different treatments, all dentists and dermatologists should have knowledge about the disease entity and the serological diagnostic methods for various types of MMP.
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integrin β4 is a major target antigen in pure ocular Mucous Membrane Pemphigoid
European Journal of Dermatology, 2016Co-Authors: Hua Qia, Hiroshi Koga, Ryosuke Sogame, Yoshiaki Hirako, Daisuke Tsuruta, Norito Ishii, Atsunari Tsuchisaka, Akiko FukumotoAbstract:Background 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.
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integrin β4 is a major target antigen in pure ocular Mucous Membrane Pemphigoid
European Journal of Dermatology, 2016Co-Authors: Hua Qian, Hiroshi Koga, Ryosuke Sogame, Yoshiaki Hirako, Daisuke Tsuruta, Norito Ishii, Atsunari Tsuchisaka, Zhexiong Jin, Kazuo TsubotaAbstract: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. Using a hemidesmosome-rich fraction, sera from patients with pure OMMP demonstrated reactivity of IgG and/or IgA antibodies to integrin β4, BP180 and laminin-332. The reactivity of pure OMMP sera to integrin β4 was further confirmed by immunoblotting using integrin β4 recombinant proteins. Using concentrated supernatant of HaCaT cells, only one serum sample showed positive IgG and IgA reactivity to LAD-1, the ectodomain of BP180. None of the pure OMMP sera reacted with any autoantigens on immunoblotting using normal human epidermal or dermal extracts, or purified human laminin-332. Integrin β4 was considered to be the major and specific autoantigen for pure OMMP. The new methods established in this study are useful for detection of various autoantigens, particularly integrin β4.
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diagnosis of oral Mucous Membrane Pemphigoid by means of combined serologic testing
Oral Surgery Oral Medicine Oral Pathology and Oral Radiology, 2014Co-Authors: Daisuke Tsuruta, Norito Ishii, Taihei Hayakawa, Minao Furumura, Hideo Fukano, Takahiro Hamada, Chika Ohata, Kazuo ShimozatoAbstract:Objective. Mucous Membrane Pemphigoid (MMP) is a rare autoimmune bullous disease caused by various autoantibodies. This study aimed to evaluate the diagnostic value of MMP-specific autoantibodies in patient sera. Study Design. We analyzed sera from 30 MMP-suspected patients with intractable oral mucosal lesions using a combination of indirect immunofluorescence with 1M NaClesplit skin, immunoblot analysis, and ELISAs. We also analyzed clinical features among different types of MMP. Results. Seventeen, 4, and 3 patients were diagnosed with anti-BP180-type MMP, anti-laminin-332-type MMP, and combined anti-BP180/anti-laminin-332-type MMP, respectively. Conclusions. Our results indicated that a combination of immunologic testing for circulating autoantibodies is useful for the diagnosis of MMP. (Oral Surg Oral Med Oral Pathol Oral Radiol 2014;117:483-496) Autoimmune bullous diseases (ABDs) are a group of heterogeneous blistering disorders that may involve the oral cavity. 1,2 ABDs are divided into the pemphigus group and subepidermal bullous disease group. Current classification of ABDs with their autoantigens is summarized in Table I. Major diseases of the pemphigus group are pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP). The subepidermal bullous disease group includes bullous Pemphigoid (BP), Mucous Membrane Pemphigoid (MMP), and epidermolysis bullosa acquisita (EBA). ABDs show autoantibodies to various epithelial adhesion molecules or extracellular matrices. Pemphigus diseases are characterized by the presence of autoantibodies against components of cell-cell adhesion, whereas Pemphigoid diseases have autoantibodies against components at the dermoepidermal junction (Figure 1). Recently developed assays for autoantibodies and autoantigens enable serologic diagnosis in 90% of ABD cases. 3-9
L S Cha - One of the best experts on this subject based on the ideXlab platform.
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urban legend series Mucous Membrane Pemphigoid
Oral Diseases, 2014Co-Authors: Giovanni Di Zenzo, Marco Carrozzo, L S ChaAbstract:Mucous Membrane Pemphigoid (MMP) is a heterogeneous group of autoimmune subepithelial blistering diseases affecting primarily Mucous Membranes showing marked degree of clinical and immunological variability. We investigated four controversial topics: (i) Does oral Pemphigoid (OP) really exist as a separate entity? (ii) Is Mucous Membrane Pemphigoid curable? (iii) What is the best therapeutic option for MMP? (iv) Does exclusive oral IgA dermatitis exist as a distinct entity from MMP? Results from extensive literature searches suggested that (i) it is still unclear whether patients with OP could be considered as a distinct subset of MMP with specific clinical and immunological features; (ii) it is uncertain whether treatment regimens that get MMP under control can be eliminated to allow patients to be in drug-free remission or they should be continuously administered in some capacities; (iii) there is a concerning paucity of good-quality trials on MMP and available recommendations are solely based on generally small patients' cohorts or case series. Some of the 2002 consensus experts' opinions should be possibly updated, particularly regarding the safety of sulfa drugs; (iv) we did not find any strong evidence to support an exclusive oral (and perhaps also mucosal) form of LAD as a separate entity.
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ocular and oral Mucous Membrane Pemphigoid cicatricial Pemphigoid
Clinics in Dermatology, 2012Co-Authors: L S ChaAbstract:Mucous Membrane Pemphigoid, a heterogeneous group of autoimmune blistering diseases, affects primarily the Mucous Membranes. Although oral and ocular mucosae can both be affected in a given patient, patients with involvement restricted to oral mucosae tend to have a benign outcome, whereas those with ocular disease commonly face treatment resistance, resulting in scarring and blindness. Diagnosis requires direct immunofluorescence microscopy to demonstrate a linear deposition of immunoglobulin (Ig) G or IgA, or complement component 3 (C3), at the epithelial basement Membrane. Although the target antigens vary, subsets of patients affected exclusively by oral and ocular mucosal diseases have autoantibodies targeting α-6 and β-4 integrins, respectively.
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the first international consensus on Mucous Membrane Pemphigoid definition diagnostic criteria pathogenic factors medical treatment and prognostic indicators
Archives of Dermatology, 2002Co-Authors: L S Cha, Stephe C Foste, Grant James Anhal, W Ernaue, Kevi D Coope, Mark J Elde, Jodavid Fine, Razzaque A Ahmed, Reza F GhohestaniAbstract:Objective We aimed to develop consensus-based recommendations for streamlining medical communication among various health care professionals, to improve accuracy of diagnosis and treatment, and to facilitate future investigations for Mucous Membrane Pemphigoid. Participants Because of the highly specific nature of this group of diseases, the 26 invited participants included either international scholars in the field of Mucous Membrane Pemphigoid or experts in cutaneous pharmacology representing the 3 medical disciplines ophthalmology, oral medicine, and dermatology. Evidence The first author (L.S.C.) conducted a literature search. Based on the information obtained, international experts who had contributed to the literature in the clinical care, diagnosis, and laboratory investigation for Mucous Membrane Pemphigoid were invited to participate in a consensus meeting aimed at developing a consensus statement. Consensus Process A consensus meeting was convened and conducted on May 10, 1999, in Chicago, Ill, to discuss the relevant issues. The first author drafted the statement based on the consensus developed at the meeting and the participants' written comments. The draft was submitted to all participants for 3 separate rounds of review, and disagreements were reconciled based on literature evidence. The third and final statement incorporated all relevant evidence obtained in the literature search and the consensus developed by the participants. The final statement was approved and endorsed by all 26 participants. Conclusions Specific consensus-based recommendations were made regarding the definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators for Mucous Membrane Pemphigoid. A system of standard reporting for these patients was proposed to facilitate a uniform data collection.
Jacqueline M Plemons - One of the best experts on this subject based on the ideXlab platform.
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a comparison of the periodontal status in patients with Mucous Membrane Pemphigoid a 5 year follow up
Journal of Periodontology, 2009Co-Authors: Austin E Schellinck, Terry D Rees, Jacqueline M Plemons, Harvey P Kessler, Francisco Riverahidalgo, Eric S SolomonAbstract:Background: Mucous Membrane Pemphigoid (MMP) is a heterogenous group of blistering autoimmune disorders of unknown etiology. Intraoral manifestations of MMP feature the formation of vesiculobullous lesions that eventually rupture, leading to pseudoMembrane-covered, irregularly-shaped ulcerations. The presence of these often painful oral lesions may hinder oral hygiene efforts resulting in increased plaque accumulation and may increase the risk of developing periodontal disease. The purpose of this study was to evaluate the changes in periodontal status in patients with MMP after a period of 5 years.Methods: Twenty patients, 10 diagnosed with MMP and 10 controls matched for age, gender, and smoking history, were chosen to participate in a study evaluating their periodontal status. Parameters evaluated included the plaque index, gingival index, bleeding index, probing depths, recession, clinical attachment level, mobility, furcation involvement, number of missing teeth, and periodontitis. Data from the same...
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periodontal status in patients with gingival Mucous Membrane Pemphigoid
Journal of Periodontology, 2006Co-Authors: Melissa B Tricamo, Terry D Rees, William W Hallmon, John M Wright, Marco A Cueva, Jacqueline M PlemonsAbstract:Background: Mucous Membrane Pemphigoid (MMP) describes an autoimmune disease with a range of variants, including those with oral, ocular, and cutaneous lesions. Persistent oral lesions, lack of effective oral hygiene, and plaque accumulation may increase the risk of long-term periodontal disease. However, to our knowledge, no studies have evaluated the relationship between the presence of gingival MMP and periodontal health. The purpose of this study was to evaluate the possible impact of gingival Pemphigoid lesions on the human periodontium.Methods: Twenty patients with histologic confirmation of subepithelial separation with or without direct immunofluorescence testing were randomly chosen to participate in a study evaluating their periodontal status. Parameters evaluated included plaque score, gingival index, bleeding index, probing depths, recession, clinical attachment level, mobility, furcation involvement, number of missing teeth, and Machtei criteria, a previously defined criteria for periodontiti...
Frederic Cau - One of the best experts on this subject based on the ideXlab platform.
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oesophageal involvement in 26 consecutive patients with Mucous Membrane Pemphigoid
British Journal of Dermatology, 2017Co-Authors: O Zehou, M Alexandre, Michel Helle, Nicole Lievre, Le C Rouxville, J J Raynaud, Gheorghe Airinei, L Laroche, F Pascal, Frederic CauAbstract:SummaryBackground Oesophageal involvement of Mucous Membrane Pemphigoid (MMP) has not yet been thoroughly described. Objectives To characterize systematically the endoscopic lesions of a series of patients with oesophageal symptoms seen at a referral centre for autoimmune bullous diseases. Methods Clinical, endoscopic and immunological findings of consecutively referred patients with MMP with oesophageal involvement, systemic and endoscopic treatments, and follow-up are described. Results Of 477 consecutive patients with MMP consulting between 2002 and 2012, 26 (5·4%) had symptomatic oesophageal involvement. Dysphagia, observed in 23 (88%) patients, was the most frequent symptom. Oesophageal symptoms could be the first sign of MMP. Patients with oesophageal involvement had a mean of three other involved sites. At initial oesophageal endoscopy, 17 of 26 patients had active lesions (intact bullae, erosions and/or erythema), 15 had stricture(s) and 12 had other cicatricial lesions. Systemic therapy alone achieved oesophageal symptom relief for five patients. Dilatation was combined with systemic therapy for 12 patients and was successful in nine; one perforation occurred. Conclusions Symptomatic oesophageal involvement affected 5·4% of patients with MMP. Dermatologists and gastroenterologists should be aware of these mucocutaneous diseases and their oesophageal involvement, as it could lead to earlier diagnosis and better care. Oesophageal dilatation could be a therapeutic option for symptomatic stricture not relieved by optimized systemic therapy alone.
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definitions and outcome measures for Mucous Membrane Pemphigoid recommendations of an international panel of experts
Journal of The American Academy of Dermatology, 2015Co-Authors: Dedee F Murrell, Anka Marinovic, Frederic Cau, Razzaque Ahmed, Katarzyna Wozniak, Joha W Aue, Stefa Eisse, Carole Pros, Luca OrradoriAbstract:Mucous Membrane Pemphigoid encompasses a group of autoimmune bullous diseases with a similar phenotype characterized by subepithelial blisters, erosions, and scarring of Mucous Membranes, skin, or both. Although knowledge about autoimmune bullous disease is increasing, there is often a lack of clear definitions of disease, outcome measures, and therapeutic end points. With clearer definitions and outcome measures, it is possible to directly compare the results and data from various studies using meta-analyses. This consensus statement provides accurate and reproducible definitions for disease extent, activity, outcome measures, end points, and therapeutic response for Mucous Membrane Pemphigoid and proposes a disease extent score, the Mucous Membrane Pemphigoid Disease Area Index.
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oral cyclophosphamide without corticosteroids to treat Mucous Membrane Pemphigoid
British Journal of Dermatology, 2013Co-Authors: E M Munyangango, M Alexandre, S Doa, Isaac Soued, F Aucouturie, Michel Helle, Nicole Lievre, Le C Rouxville, F Pascal, Frederic CauAbstract:Summary Background Mucous Membrane Pemphigoid (MMP) still represents a potentially life- and sight-threatening disease. Immunosuppressants, such as cyclophosphamide (CYC), are indicated for patients with severe and/or refractory MMP. Objectives To evaluate the efficacy and safety of daily oral CYC without corticosteroids as therapy for severe MMP. Methods Thirteen patients with severe refractory MMP, who received oral CYC at an initial dose of 2 mg kg−1 without corticosteroids, were retained. Previous treatments, for example dapsone, sulfasalazine or topical agents, were maintained during CYC treatment. Initial clinical severity and response to treatment were assessed by scoring. CYC was stopped after complete remission (CR), or when MMP progressed or lymphopenia (< 0·7 × 109 cells L−1) occurred. Results After 52 weeks of CYC treatment, the overall response rate was 69% (9/13 patients) with a median time to disease control of 8 weeks (range 4–52 weeks). Seven patients (54%) entered CR with a median time to CR of 24 weeks (range 16–52 weeks), all remaining in CR at week 52. The mean duration of CYC administration was 12 weeks (range 2–52 weeks). The most common side effect was lymphopenia (10/13 patients), which led to CYC withdrawal for six patients. No sepsis was observed. Conclusions CYC without corticosteroids had rapid efficacy in patients with severe refractory MMP and was safe.
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rituximab for patients with refractory Mucous Membrane Pemphigoid
Archives of Dermatology, 2011Co-Authors: Christelle Le Rouxville, Frederic Cau, C Prostsquarcioni, M Alexandre, S Doa, Mariedominique Ette, Isaac Soued, E Gabiso, F Pascal, F AucouturieAbstract:Background Mucous Membrane Pemphigoid (MMP) still represents a potentially life- and sight-threatening disease. In a subset of patients with severe MMP, conventional immunosuppressants are ineffective or contraindicated. Observations Twenty-five patients with severe refractory MMP, including 5 with Mucous Membrane –dominant epidermolysis bullosa acquisita, received 1 or 2 cycles of rituximab (375 mg/m 2 weekly for 4 weeks). Twenty-one of the patients were receiving concomitant therapy with dapsone and/or sulfasalazine therapy, which was maintained during rituximab cycles. Complete responses in all affected sites (ocular and/or extraocular) were obtained in 17 patients (68%) by a median time of 12 weeks after the first cycle, and 5 additional patients responded completely after a second cycle, yielding an 88% complete response rate. In all but 1 of the 10 patients with ocular lesions, their eyes became noninflammatory within a mean of 10 weeks. Among the 3 patients (12%) who developed severe infectious complications, 2 (8%) died; they had been receiving concomitant conventional immunosuppressants and high-dose corticosteroids and were hypogammaglobulinemic. Treatment with immunosuppressants was discontinued for all other patients, and no other infection was observed. Ten patients experienced relapse after a mean of 4 (range, 1-16) months after achieving complete responses. Conclusions Rituximab appears to have rapid and dramatic efficacy in patients with severe, refractory MMP. The occurrence of severe infections in patients receiving concomitant conventional immunosuppressants supports using rituximab without other immunosuppressants. Controlled prospective studies are warranted to define an optimal treatment protocol.