Diagnosis Of Mucous Membrane Pemphigoid Research Articles

Evaluation of the Diagnostic Value of Oesophageal Biopsies for Direct Immunofluorescence Microscopy in Mucous Membrane Pemphigoid.

Mucous membrane pemphigoid is an autoimmune blistering disorder characterized by predominant involvement of surface-close epithelia and linear depositions of immunoreactants at the dermal-epithelial junction on direct immunofluorescence microscopy. A major diagnostic difficulty is the frequent need for multiple biopsies to facilitate the diagnosis. Although oesophageal involvement is a rare, but life-threatening manifestation, the relevance of oesophageal direct immunofluorescence sampling is unclear. This retrospective monocentric study evaluated 67 non-lesional biopsies from 11 patients with mucous membrane pemphigoid and clinical symptoms suggestive of oesophageal involvement, comprising 31 samples from the oesophagus and 36 samples from other anatomical sites. Five patients (45.5%) exhibited endoscopic findings compatible with oesophageal involvement of mucous membrane pemphigoid. No correlation was identified between the presence of oesophageal lesions and direct immunofluorescence positivity in lesions from the oesophagus (p = 1.0). Oral and cutaneous samples were significantly more frequently positive by direct immunofluorescence than were oesophageal biopsies (p < 0.0001 and p = 0.0195, respectively). Oesophageal samples yielded significantly less IgG reactivity than oral and cutaneous lesions (p < 0.0001 and p = 0.0126, respectively), and less IgA antibody response than oral lesions (p = 0.0036). In conclusion, oesophageal direct immunofluorescence samples were inferior to oral and cutaneous biopsies for the diagnosis of mucous membrane pemphigoid even when oesophageal lesions compatible with mucous membrane pemphigoid were present at the time of biopsy.

Long-term outcomes of fornix reconstruction and cicatricial entropion repair in ocular mucous membrane pemphigoid and drug-related secondary pemphigoid

ObjectifExaminer les résultats à long terme de la reconstruction du fornix oculaire et de la réparation de l'entropion cicatriciel en présence d'une pemphigoïde de la muqueuse oculaire (MMP, pour mucous membrane pemphigoid) et d'une MMP secondaire. MéthodesOn a procédé à un examen rétrospectif des dossiers médicaux de patients qui présentaient une MMP et qui devaient subir une reconstruction du fornix oculaire (greffe de membrane amniotique ou greffe de muqueuse buccale) ou une réparation de l'entropion cicatriciel (intervention de Wies) entre le 1er janvier 2000 et le 1er septembre 2020. La biopsie de la muqueuse des patients était positive et/ou ces derniers présentaient des caractéristiques cliniques typiques d'une MMP ou d'une MMP secondaire. Le paramètre primaire consistait en une réussite globale de la reconstruction du fornix oculaire mesurée par le maintien de sa profondeur lors de la dernière visite de suivi. La disparition du trichiasis, l'acuité visuelle et le soulagement des symptômes subjectifs comptaient au nombre des paramètres secondaires. RésultatsAinsi, 8 patients (10 yeux) présentant un diagnostic de MMP (3 hommes et 5 femmes; âge médian : 71 ans) et 4 patients (4 yeux) présentant un diagnostic de MMP secondaire (2 femmes et 2 hommes; âge médian : 87 ans) ont été admis à l’étude. La durée moyenne du suivi était de 22,7 mois (fourchette : 0,3–87,5 mois) dans le cas des patients présentant une MMP et de 15,4 mois (fourchette : 3,0–43,9 mois) dans celui des patients présentant une MMP secondaire. Dans le groupe MMP, 30,0 % des yeux ont fait l'objet d'une reconstruction du fornix oculaire, 60,0 % des yeux ont fait l'objet d'une réparation de l'entropion et 10,0 % des yeux ont subi les 2 formes d'intervention. La récurrence d'un symblépharon et la perte de la profondeur du fornix oculaire sont apparues dans tous les yeux qui présentaient une MMP en moyenne 6,4 ± 7,0 mois après l'intervention; le trichiasis avait réapparu chez tous les patients lors de la dernière visite de suivi. Chez les patients qui présentaient une MMP secondaire, 75,0 % des yeux ont présenté une récurrence du symblépharon, et 66,7 %, une récurrence du trichiasis. On a noté un soulagement à court terme des symptômes tant dans la MMP que dans la MMP secondaire. ConclusionsLa reconstruction du fornix oculaire et la réparation de l'entropion cicatriciel au sein de notre cohorte de patients présentant une MMP ou une MMP secondaire ont donné lieu à un soulagement à court terme des symptômes, mais une récurrence s'est produite en moyenne 6 mois après l'intervention.

Refractory esophageal stricture of esophageal mucous membrane pemphigoid after allogeneic hematopoietic stem cell transplantation

Haploidentical allogeneic hematopoietic stem cell transplantation from her brother was performed on a 41-year-old lady with no prior history of pemphigoid to treat recurrent AML. On day 59 following transplantation, she experienced esophageal stenosis. During immunosuppressive therapy for graft vs. host disease, this condition was controlled with periodic esophageal dilatation (GVHD). Her esophageal stricture, which required periodic dilatation, grew worse after she stopped immunosuppressive therapy because of recurrent AML. The esophageal mucosa was easily hemorrhagic and desquamative. Histologic analysis revealed that the squamous cell layers had been divided. Indirect immunofluorescence was negative for IgG and positive for IgA on the epidermal layers, while direct immunofluorescence showed a linear deposition of IgG on the basement membrane zone. It was determined through immunoblotting utilizing recombinant protein of BP180 C-terminal domain that both IgG and IgA antibodies were present, supporting the diagnosis of mucous membrane pemphigoid with anti-BP180. After allogeneic transplantation, basal epidermal cell destruction by GVHD may result in autoimmune blistering disorders, which expose basement membrane proteins and antigen presentation. A similar mechanism could apply to our situation. For rare GVHD cases, a thorough histological diagnosis is required.

Mucous Membrane Pemphigoid: A Case Report with Oral and Ocular Presentation

To describe the diagnosis and management of mucous membrane pemphigoid (MMP) with oral and ocular presentation. Mucous membrane pemphigoid constitutes a heterogeneous group of chronic, autoimmune vesiculobullous diseases characterized by blister formation that has a propensity to affect different mucous membranes of the body. The most commonly affected areas include the oral cavity, mucous membranes of the eyes, throat, genitalia, and nose. This disease usually affects elderly women with a peak incidence at around 50-70 years of age; however, rare cases have been diagnosed in children. The symptoms of MMP include recurrent blistering lesions which eventually rupture and occasionally heal with scarring that may lead to certain complications involving the eyes and throat regions. In this report, we describe a 66-year-old female patient who complained of oral and ocular lesions for a period of 2 years. Pain, burning mouth, and gingival inflammation were present. Ocular examination showed mild conjunctivitis with scar formation at the lateral canthus of the left eye. The patient also noticed periods of water-filled balloon-like formation in the gingiva that rupture spontaneously leaving sore spots. A biopsy was obtained from perilesional tissue and sent for histopathological examination, correlation of clinical and histological features directed us toward the diagnosis of MMP. The patient was treated for both oral and ocular lesions using topical corticosteroid therapy in conjunction with antifungal and antibacterial drugs. The response to local treatment was augmented via effective periodontal therapy to control the concurrent plaque-induced gingival inflammation and via using a customized application tray to sustain the drug efficacy. A multidisciplinary approach is often necessary in order to treat MMP lesions efficaciously. Early diagnosis and effective treatment protocol using systemic or topical corticosteroid therapy along with other therapeutic means including periodontal therapy, good oral hygiene practice, and timely follow-up are very useful in preventing long-term complications due to this disease.

Assessment of Diagnostic Strategy for Mucous Membrane Pemphigoid

An accurate diagnosis of mucous membrane pemphigoid (MMP) is essential to reduce diagnostic and therapeutic delay. To assess the diagnostic accuracy of direct immunofluorescence microscopy on mucosal biopsy specimens and immunoserology in a large cohort of patients with suspected MMP. This retrospective cohort study was carried out in a single tertiary care center for blistering diseases between January 2002 and March 2019. Eligible participants were patients with suspected MMP and paired data on at least a mucosal biopsy specimen for direct immunofluorescence microscopy (DIF) and indirect immunofluorescence microscopy (IIF) on a human salt-split skin substrate (SSS). In addition, an optional DIF test on a skin biopsy specimen and one or more performed routine immunoserologic tests were analyzed. Data analysis was conducted from April 2019, to June 2020. Diagnostic accuracy of DIF, IIF SSS, and immunoblot for BP180 and BP230. Of the 787 participants, 121 (15.4%) received the diagnosis of MMP (50 men [41.3%], 71 women [58.7%]; mean [SD] age at diagnosis, 60.1 [17.7] years). Sixty-seven of the patients with MMP (55.4%) had monosite involvement, of which oral site was the most frequently affected (51 [42.1%]). No significant difference was found between the sensitivity of DIF on a perilesional buccal biopsy and a normal buccal biopsy (89.3% vs 76.7%). Three patients with solitary ocular involvement showed a positive DIF of only the oral mucosa. In 6 patients with a negative mucosal DIF, a skin biopsy confirmed diagnosis of MMP. Overall, IIF SSS was less sensitive (44.6%), but highly specific (98.9%). The sensitivity of immunoblot (66.1%) was higher compared to SSS, but with lower specificity (91.3%). This comparative diagnostic accuracy study of a cohort of 787 patients found a high sensitivity of a mucosal DIF biopsy for diagnosis of MMP, and lower sensitivity of serologic analysis. A biopsy can be taken from either perilesional or normal buccal mucosa. An additional DIF biopsy of another mucosal site or of affected or unaffected skin may increase the diagnostic yield and is recommended in patients with negative DIF results and high clinical suspicion.

Autoantibody Detection for Diagnosis in Direct Immunofluorescence-Negative Mucous Membrane Pemphigoid: Ocular and Other Sites Compared

To assess whether a panel of serum pemphigoid autoantibody tests could be used to confirm an immunopathologic diagnosis of mucous membrane pemphigoid (MMP) in direct immunofluorescent negative (DIF-) MMP patients. Prospective cross-sectional study. Seventy-six patients with multisite MMP with 45 matched control participants. Enzyme-linked immunosorbent assays (ELISAs) for BP180 and BP230 (MBL International), immunoglobulin A (IgA) A and immunoglobulin G indirect immunofluorescence (IIF) on human salt-split skin and the keratinocyte footprint assay for anti-laminin 332 antibodies. Sensitivity and specificity of autoantibody detection and significant differences for individual tests and test combinations for MMP involving different sites. All DIF- patients (24/73 [31.8%]) had either ocular-only disease or ocular involvement in multisite disease. Serum pemphigoid autoantibodies were detected in 29 of 76 MMP patients (38.2%) compared with 3 of 45 control participants (6.7%). Autoantibody reactivity detected by any 1 or more of the tests was present in 6 of 24 DIF- patients (25%) compared with 22 of 49 DIF positive (DIF+) patients (44.9%). Ocular-only MMP serum reactivity was not significantly different for any test or test combination compared with control participants, whereas DIF- multisite ocular MMP differed for 1 ELISA and 3 of 7 test combinations. By contrast, for DIF+ nonocular MMP patients, all the individual tests, apart from IgA IIF, and all test combinations were significantly different compared with those for control participants. For the entire MMP cohort, the sensitivity of all individual tests was low, having a maximum of 21.05% for BP180 reactivity but increasing to 38.16% for an optimal test combination. Disease activity was associated strongly with positive serologic findings. Pemphigoid serum autoantibody tests did not provide immunopathologic evidence of MMP in ocular-only MMP patients but showed limited value in DIF- multisite ocular MMP patients. The requirement for immunopathologic confirmation of MMP by autoantibody detection is inappropriate for DIF- ocular-only MMP patients, resulting in missed diagnoses, delayed therapy, and poor outcomes. Alternative diagnostic criteria for ocular-only MMP are required to exclude the other causes of scarring conjunctivitis until more sensitive and specific immunopathologic tests become available.

A study into the best sites to biopsy for a diagnosis of pemphigus vulgaris or mucous membrane pemphigoid

Pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) are rare and potentially serious conditions that cause painful blisters or erosions to develop in the mouth, nose, throat, oesophagus, genitalia and skin. One of the main complications unique to MMP is that it may scar affected sites, particularly the eyes. Both diseases generally occur in older adults. To confirm the diagnosis of PV and MMP, a biopsy has traditionally been taken from near the affected area (perilesional). Where only mucosal surfaces are involved, the mouth is the most accessible site, however this can be difficult because the lining of the mouth is fragile. The aim of this study was to compare outcomes of a biopsy of normal healthy-looking tissue lining the inner cheek (buccal mucosa) with a perilesional biopsy. The authors, based in the U.K., analysed the biopsy location and results of 251 MMP and 77 PV patients. They have shown that a biopsy of normal buccal mucosa tissue is as good as a perilesional biopsy for diagnosis of oral PV. For MMP patients with multiple oral surfaces affected, biopsy of normal buccal mucosa was almost equal to taking a perilesional sample for diagnosis. For MMP patients with only the gums affected, neither a perilesional biopsy from the gum nor a normal buccal mucosal biopsy were reliable enough to always confirm the diagnosis. Importantly for this group of patients, the authors have shown that a biopsy from the junction between the lining of the cheek and gum gave a positive result in all cases. A punch biopsy taken from normal buccal mucosa tissue is a simple and safe practical method for diagnosing all patients with oral PV and all MMP patients, with the exception of those with gum only lesions. This is a summary of the study: The optimal oral biopsy site for diagnosis of mucous membrane pemphigoid and pemphigus vulgaris

FABRY DISEASE: ORAL FINDINGS AND POSSIBLE ASSOCIATION WITH AUTOIMMUNE DESQUAMATIVE GINGIVITIS

Fabry disease is an X-linked metabolic disease caused by the deficiency of the lysosomal enzyme α-galactosidase A. We report a case of a 55-year-old woman, with previous diagnosis of Fabry disease that was referred complaining of pain in the gingiva for about 1 year. The physical examination revealed short fingers, angiokeratomas in the neck, fissured tongue and desquamative gingivitis. An incisional biopsy was performed in the gingival lesions and the histopathologic analysis revealed a subepithelial blister, compatible with the diagnosis of mucous membrane pemphigoid. A topical corticosteroid therapy was instituted associated with conventional periodontal treatment. After 3 weeks, all areas of erythema or ulceration were healed, and the symptoms had disappeared. Fabry disease is a rare disorder with a few cases of oral abnormalities described. A high incidence of autoantibodies has been reported in patients with Fabry disease, which may explain the development of the desquamative gingivitis condition. Fabry disease is an X-linked metabolic disease caused by the deficiency of the lysosomal enzyme α-galactosidase A. We report a case of a 55-year-old woman, with previous diagnosis of Fabry disease that was referred complaining of pain in the gingiva for about 1 year. The physical examination revealed short fingers, angiokeratomas in the neck, fissured tongue and desquamative gingivitis. An incisional biopsy was performed in the gingival lesions and the histopathologic analysis revealed a subepithelial blister, compatible with the diagnosis of mucous membrane pemphigoid. A topical corticosteroid therapy was instituted associated with conventional periodontal treatment. After 3 weeks, all areas of erythema or ulceration were healed, and the symptoms had disappeared. Fabry disease is a rare disorder with a few cases of oral abnormalities described. A high incidence of autoantibodies has been reported in patients with Fabry disease, which may explain the development of the desquamative gingivitis condition.

TREATMENT OF MUCOUS MEMBRANE PEMPHIGOID WITH LOW-LEVEL LASER THERAPY

Pemphigoid of the mucous membranes represents a group of subepithelial blistering diseases of autoimmune origin. The radiation emitted by the low-level laser affects the metabolic processes of the target cells, producing biostimulants that act in the process of regeneration of the tissues. A 34-year-old female patient presented clinically with an erythematous gingiva that was blistering and scaling, with painful and burning symptomatology. An excisional biopsy was performed, sent for histopathologic examination, confirming the diagnosis of mucous membrane pemphigoid. Treatment included low-level laser therapy at the wavelength 630 to 700 nm, following the 1 J protocol for interdental papilla, with an area of approximately 1 cm² per point, and use of steroids. The objective of this study is to demonstrate how laser therapy functions in the treatment of oral lesions of mucous membrane pemphigoid. Pemphigoid of the mucous membranes represents a group of subepithelial blistering diseases of autoimmune origin. The radiation emitted by the low-level laser affects the metabolic processes of the target cells, producing biostimulants that act in the process of regeneration of the tissues. A 34-year-old female patient presented clinically with an erythematous gingiva that was blistering and scaling, with painful and burning symptomatology. An excisional biopsy was performed, sent for histopathologic examination, confirming the diagnosis of mucous membrane pemphigoid. Treatment included low-level laser therapy at the wavelength 630 to 700 nm, following the 1 J protocol for interdental papilla, with an area of approximately 1 cm² per point, and use of steroids. The objective of this study is to demonstrate how laser therapy functions in the treatment of oral lesions of mucous membrane pemphigoid.

Detection of IgE autoantibodies in mucous membrane pemphigoid and their association with disease severity.

Mucous membrane pemphigoid (MMP) is an autoimmune disease characterized by scarring lesions at mucosal sites. Although the pathogenic role of specific IgG and/or IgA has been already demonstrated and the detection of these immunoglobulins is a criterion in the diagnosis of MMP, little is known about IgE role in this disease. Therefore, the main purpose of this study was to assess the presence of circulating and tissue bound IgE in patients with MMP and their possible correlations with clinical presentation and disease course. We conducted a retrospective study on 29 patients affected by MMP, recruited from a single center. Direct and indirect immunofluorescence studies were assessed to analyze the presence of specific IgE directed against the basal membrane zone. For each patient, fluorescence data were compared to clinical features. Linear deposits of C3, IgG and IgA were present in 86.2%, 62% and 37.9% of cases respectively, while IgE linear deposits were detected in 17 out of 29 patients (58.6%) including one case with isolated IgE positivity. Circulating IgE and IgA anti-BMZ were present in 7 (24.1%) and 5 (17.2%) patients, respectively. Both the presence of circulating IgA and of tissue bound IgE deposits correlated with disease activity index (P<0.014). Our results demonstrated the presence of IgE autoantibodies in MMP, particularly in more severe cases. Thus, IgE detection may represent an additional useful diagnostic tool in this disease.

A Challenging in Diagnosis of Mucous Membrane Pemphigoid with Desquamative Gingivitis Presentation: A Case Report

: Desquamative gingivitis (DG) is a descriptive term, indicating inflamed and peeling gingiva. It is characterized by sloughing of the gingival epithelium as a result of formation and rupture of the vesicle. Consequently, the gingiva appears erythematous and often edematous. Desquamative gingivitis may represent an oral clinical manifestation of various systemic mucocutaneous diseases. This case report is on a 42-year-old female with chief complaint of burning sensation in the anterior maxillary gingiva. The patient did not have any other lesion at other sites of the oral cavity. The patient had been affected by asthma and her medications were salbutamol spray and tab cetirizine. Clinically, anterior gingiva appeared as the presentation of desquamative gingivitis. Therefore, an incisional biopsy of the affected area for histopathologic assessment and an incisional biopsy of clinically normal-appearing gingiva for direct immunofluorescence (DIF) study were performed. The histopathologic report was herpes simplex infection. However, this report was incompatible with the clinical view and the patient history. Therefore, a second biopsy was performed. The second histopathologic report was compatible with MMP. The treatment plan was local corticosteroid with follow-up. At the one-month examination visit, a significant improvement was observed in the clinical view of the gums.

Mucous Membrane Pemphigoid with Ocular Involvement: The Clinical Phenotype and Its Relationship to Direct Immunofluorescence Findings

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. Prospective, cross-sectional study. Patients meeting the clinical criteria of ocular MMP, including those with positive and negative DIF findings. A case report form was used to collect the demographic details, the clinical history, and the results of a detailed clinical assessment by ophthalmologists, otolaryngologists, dermatologists, and oral medicine specialists. All anatomic sites potentially affected by MMP were examined apart from the esophagus (and larynx in a subset). The DIF results were recorded. Differences between DIF-positive and -negative patients in demography, sites of involvement, and disease severity as determined by the degree of conjunctival scarring (using Tauber staging), central corneal disease (vascularization, scarring, ulceration, and conjunctivalization), history of conjunctival or lid surgery, and requirement for systemic immunotherapy at the time of screening. A total of 73 patients with ocular MMP were recruited, of whom 20 of 73 (27.4%) had ocular-only disease. There was no significant demographic or clinical difference between patients with positive and negative DIF results. This finding included differences in disease severity for which the only significant difference was that of more severe central corneal disease in DIF-negative patients. Asymptomatic disease at different sites was frequent. These findings do not support the classification of DIF-negative patients, meeting the clinical criteria for ocular MMP, as having a different disease. This category of patients should be accepted as having DIF-negative MMP, for clinical management purposes, with patients having inflamed eyes being treated with systemic immunomodulatory therapy. The frequent finding of asymptomatic ocular, oral, and nasopharyngeal MMP is clinically significant and implies that these sites should be routinely screened in asymptomatic patients.

Drug-induced oral mucous membrane pemphigoid: a case report

Observation: An 82-year-old patient treated with the antihistamine ebastine (Kestin ® ) for allergic rhinitis presented for gingival pain. The diagnosis of mucous membrane pemphigoid was suspected after a positive pinch test. Blood examinations highlighted AC anti-PBGA2, and the gingival biopsy showed an epithelioconjunctival delamination and the presence of anti-IgA antibody and C3 deposition along the basal of the membrane. Topical corticosteroid administration was initiated. The symptoms improved but elevated IgA and C3 levels persisted. He underwent a complete remission on cessation of ebastine treatment. Commentary: Mucous membrane pemphigoid is an autoimmune disease whose origin remains unclear. The aim of the treatment is to decrease the symptoms, but full recovery of the patients is exceptional. We report here a clinical case with symptom resolution because of the ebastine withdrawal. Ebastine can thus be suspected as a cause if a patient presents with oral symptoms of mucous membrane pemphigoid.

Multiple and repeated sampling increases the sensitivity of direct immunofluorescence testing for the diagnosis of mucous membrane pemphigoid

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. To assess the impact of multiple and repeated DIF sampling on establishing the diagnosis of MMP. We reviewed the results of DIF studies in 136 nonlesional biopsies from 78 patients who were immunologically confirmed to have MMP. Thirty-six of 52 patients (69%) who underwent only 1 biopsy at the first workup were positive. In 13 cases, the initial single biopsy was negative, and later biopsies were positive. Twenty-two of 26 patients (85%) who underwent multiple biopsies at the initial workup showed ≥1 positive DIF test result. Simultaneously obtained biopsies yielded discordant positive and negative findings in 11 patients. Overall, 74 of 78 patients (95%) had ≥1 positive result by DIF test. In the remaining 4 cases, the diagnosis was confirmed by the detection of circulating autoantibodies against BP180. This is a retrospective, single-center study. Our data demonstrate that multiple and repeated biopsies increase the sensitivity of the DIF test for MMP diagnosis. Negative DIF test findings in cases clinically suggestive of MMP should prompt repeat biopsies.

How sensitive is direct immunofluorescence for the diagnosis of mucous membrane pemphigoid?

How sensitive is direct immunofluorescence for the diagnosis of mucous membrane pemphigoid?

Clinical Course of Four Patients with Intractable Recurrent Oropharyngeal Ulcers

We report four patients with intractable recurrent oropharyngeal ulcers, including two with idiopathic oropharyngeal ulcers, one with intestinal Behcet’s disease, and one with mucous membrane pemphigoid. The diagnosis of intestinal Behcet’s disease was confirmed by gastrointestinal endoscopy, and the diagnosis of mucous membrane pemphigoid was confirmed by skin biopsy. However, no causative disease could be identified in the two cases with idiopathic oropharyngeal ulcers on the basis of any of the clinical findings, blood examinations or histological analysis. The patients with idiopathic oropharyngeal ulcers were treated by steroids combined or not combined with colchicine. The patient with intestinal Behcet’s disease was treated with infliximab. The patient with mucous membrane pemphigoid was treated using a combination of steroids with diaminodiphenyl sulfone and rituximab. During clinical observation over an average period of 7 years, all the patients have maintained a good course without a recurrence of severe ulcers.

Rituximab combined with conventional therapy versus conventional therapy alone for the treatment of mucous membrane pemphigoid (MMP)

The use of rituximab for refractory autoimmune blistering diseases is increasing. Data related to rituximab for the treatment of mucous membrane pemphigoid (MMP) are limited. We sought to compare the efficacy of adding rituximab with traditional immunosuppressive therapies in the treatment of MMP. The primary outcome was achievement and time to disease control. Patients with a diagnosis of MMP from August 2001 to June 2015 who had greater than 6 months of follow-up after the initiation of therapy were reviewed. In all, 24 patients were treated with rituximab and 25 were treated with conventional immunosuppression. Of patients, 100% in the rituximab group achieved disease control compared with 40% in the conventional group (P < .01), with a mean time to disease control of 10.17 months and 37.7 months (P = .02). Adverse events were seen in 33% of patients after rituximab, compared with 48% of patients in the conventional group (P = .2). Rituximab dosing was not uniform and the 2 groups were not matched in terms of disease severity, nor were they randomized. Our study indicates that the addition of rituximab to conventional therapy in patients with MMP results in more rapid and sustained disease control with potentially fewer adverse events.

Immunopathological Diagnosis of Mucous Membrane Pemphigoid (MMP)

Background: Mucous membrane pemphigoid (MMP) is a serious, autoimmune, blistering disorder which primarily involves the mucous membranes and that can result in blindness and other complications as a result of scarring of mucous membranes. The oral cavity and the eye are most frequently involved. Since extension of the lesion into pharynx causes dysphagia and progressive ocular lesions may cause blindness, early and valid diagnosis is very important. Diagnosis of MMP is a problem because patients afflicted are elderly, the disease is chronic, and is often in an advanced stage when the diagnosis is established and requires aggressive therapy for control. Oral and dental care is important in all phases of the diagnosis and treatment of the patient with mucous membrane pemphigoid. Early recognition and diagnosis are important for early treatment, improving survival and for limiting the complications of therapy. Methods: Here we present a case of mucous membrane pemphigoid with onset at age 49 in patient who manifested severe periodontal disease and showed the lesion only on the mucous membrane of the gingiva. We describe how we made a differential diagnosis from other diseases which also accompany similar lesions. Results: We examined the clinical manifestations, blood test results, histopathologic findings of the affected tissue, and immunological findings in relation to autoimmunity. Conclusion: We believe that the diagnostic strategy described in the present study will be quite informative for making rapid and definite diagnoses of similar cases.

Autoantibody Profile of a Cohort of 78 Italian Patients with Mucous Membrane Pemphigoid: Correlation Between Reactivity Profile and Clinical Involvement.

Direct diagnosis of mucous membrane pemphigoid (MMP) is not easy. Circulating autoantibodies targeting bullous pemphigoid antigens of 180 kDa and 230 kDa (BP180 and BP230), α6β4 integrin, laminin 332 and type VII collagen (Col VII) are not always present. The aims of this study were to characterize the humoral immune response of a cohort of Italian patients with MMP, its association with clinical involvement and severity, and to design an algorithm for efficient serological diagnosis. Seventy-eight MMP sera were studied retrospectively by indirect immunofluorescence on salt-split skin, enzyme-linked immunosorbent assay (ELISA) and immunoblotting. Indirect immunofluorescence on salt-split skin resulted in the most sensitive approach for diagnosis of MMP. BP180 was the major autoantigen in MMP patients with oral and cutaneous involvement. Significant associations were found between BP180 reactivity and oral and cutaneous localization of the lesions (p = 0.006), and between Col VII positivity and Setterfield severity score (p = 0.020).

Mucous membrane pemphigoid: a retrospective study of 68 cases

Objective To analyze the clinical features and treatment of 68 cases of mucous membrane pemphigoid (MMP).Methods Clinical data were collected from 68 patients with MMP at the Department of Dermatology,Peking University First Hospital,between August 1987 and October 2012.Skin manifestations,histopathological and immunological findings were studied,with an emphasis on treatment regimens.Results The two most frequently involved sites were oral (67/68,98.5％) and conjunctival (23/68,33.8％) mucosa in patients with MMP.Immunological examinations included direct immunofluorescence test,indirect immunofluorescence test and enzyme-linked immunosorbent assay,with a positivity rate of 50％ (8/16),20.5％ (8/39) and 53.7％ (22/41) respectively.According to lesion distribution and disease severity,patients were given local therapy (n =5) or low to moderate dose of glucocorticoids (n =55,0.4-0.5 mg/kg or 30 mg per day).The condition was controlled until the dose of glucocorticoids reached 50 mg/d in three patients with cutaneous,oral,ocular,and other mucosal involvement.The time to onset of action of glucocorticoids at the controlling dose was (11.80 ± 5.88) days,and the duration of administration of glucocorticoids at this dose varied from 0.23 to 12 months (average,3.06 ± 2.84 months).Fifteen patients were almost cured,and four patients completely cured.Of the 15 patients almost cured,the time required for a 50％ reduction in the dose of glucocorticoids was (13.29 ± 5.76) months,and that required for the control of MMP was (17.33 ± 7.71) months.The dose of glucocorticoids was decreased to 76.5％ of the controling dose at 6 months after the control of MMP,and 58.1％ of that at 12 months.Oral candidiasis occurred in three patients during the treatment.Conclusions The diagnosis of MMP is mainly based on typical clinical and histopathological findings,and current immunological examinations are insufficiently sensitive.Usually,low to moderate dose of systemic corticosteroids combined with topical therapy can lead to satisfactory treatment outcomes. Key words: Pemphigoid, benign mucous membrane; Retrospective studies; Drug therapy