Case Of Mucous Membrane Pemphigoid Research Articles

Retrospective analysis of the long-term therapeutic effectiveness and safety profile of rituximab in the treatment of mucous membrane pemphigoid in a German university center between 2008 and 2019.

The B-cell-depleting anti-CD20 antibody rituximab (RTX) is often used as an adjuvant drug for the treatment of refractory cases of mucous membrane pemphigoid (MMP). This study aims to determine the therapeutic effectiveness and the safety profile of RTX in MMP. The medical records of all cases of MMP treated with RTX between 2008 and 2019 in our university medical center located in northern Germany, which specialized in autoimmune blistering skin diseases, were retrieved and systemically analyzed for treatment responses and potential adverse events over a median period of 27 months. We identified 18 MMP patients who received at least one cycle of RTX to treat MMP. RTX was always used as an adjuvant treatment, and its application did not change concomitant treatments. Under treatment with RTX, 67% of the patients achieved an improvement in their disease activity within 6 months. This was also reflected in a statistically significant reduction in the Mucous Membrane Pemphigoid Disease Index (MMPDAI) activity score. The frequency of infections under RTX treatment increased only slightly. The use of RTX is associated with an attenuation of MMP in a large proportion of MMP patients in our study. At the same time, its application was not found to further increase the susceptibility of the most strongly immunocompromised population of MMP patients to opportunistic infections. Collectively, our results suggest that the potential benefits of RTX outweigh its risks in patients with refractory MMP.

A Case of Mucous Membrane Pemphigoid with Antibodies Against Type VII Collagen

A Case of Mucous Membrane Pemphigoid with Antibodies Against Type VII Collagen

A case of mucous membrane pemphigoid in the oral mucosa due to DPP-4 inhibitor

A case of mucous membrane pemphigoid in the oral mucosa due to DPP-4 inhibitor

Oral Mucous Membrane Pemphigoid With Positive Nikolsky’s Sign: A Case Report

Mucous membrane pemphigoid (MMP) is a rare inflammatory, autoimmune, and subepithelial vesiculobullous disease in which tissue-bound autoantibodies are produced against one or more components of the basement membrane. Oral lesions of the pemphigoid begin in the form of vesicles or bullae that often involve throughout the mouth but may be confined to specific areas, especially the gingiva, in a pattern known as desquamative gingivitis. The positive Nikolsky's sign is characteristic of pemphigus vulgaris, in which a blister can appear on the normal-appearing skin if exerting lateral pressure, and is very rare in the mucosa and other vesiculobullous diseases. Here we report a case of mucous membrane pemphigoid that developed as desquamated gingivitis in a 46-year-old woman with positive Nikolsky's sign in the gingival mucosa. In the histopathologic view, a subepithelial cleft was observed. The results of direct and indirect immunofluorescence tests and related therapeutic interventions are also presented. Positive Nikolsky's sign can be observed in the mucosa as well as in the mucous membrane pemphigoid in addition to pemphigus vulgaris, and vesiculobullous lesions should be diagnosed based on the sum of clinical, histopathological, and immunofluorescence findings.

Dermatomyositis in patients with autoimmune blistering diseases.

It is common for multiple autoimmune diseases to occur in the same patient. However, autoimmune blistering diseases (AIBD) do not commonly associate with dermatomyositis (DM). We performed a literature review and found 12 previous reports that may be attributed to misdiagnosis, underreporting, or true rarity of association. Herein, we present a case of pemphigus vulgaris and a case of mucous membrane pemphigoid associated with DM and review the related literature. AIBD-associated interstitial lung disease, genetic predisposition, potential environmental triggers of both AIBD and DM, drug-related triggers, and paraneoplastic processes are discussed. Dermatologists must be vigilant for a second autoimmune disease in patients with AIBD that may have therapeutic implications.

Oral, Pharyngeal and Laryngeal Lesions in Mucous Membrane Pemphigoid and Usefulness of Narrow Band Imaging

Mucous membrane pemphigoid is a relatively rare disorder involving mucosal lesions. Here, we report two cases of mucous membrane pemphigoid. This report is the first to discuss narrow band imaging of the mucosal features of mucous membrane pemphigoid. The two patients presented with sore throat and multiple stomatitis. Optic fiberscopy revealed mucosal lesions on the gingiva, palate, epiglottis, and arytenoid. The appearance showed both erosion and erosion with a white coating. Narrow band imaging clearly showed a white area at the site of the sub epithelial blisters and a mixed blue and brownish area at the site of the erosive area in which the mucosa was exfoliated without blistering. Narrow band imaging was therefore also useful for detecting blister lesions in mucous membrane pemphigoid because lesions were more clearly visible. Pharyngolaryngeal examination using optic fiberscopy should be performed in all mucous membrane pemphigoid patients.

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.

A Case of Mucous Membrane Pemphigoid Involving the Trachea and Bronchus with Autoantibodies to β3 Subunit of Laminin-332

Mucous membrane pemphigoid (MMP) is an autoimmune bullous disease of the mucous membranes, including the eye, nose, oral cavity, pharynx, larynx and oesophagus, with occasional skin lesions (1). We report here a case of a patient with MMP who demonstrated concomitant tracheal and bronchial lesions, in addition to oral, ocular and cutaneous lesions, in whom autoantibodies to β3 subunit of laminin-332 were detected by immunoblot analysis.

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

Childhood Mucous Membrane Pemphigoid

To report a case of mucous membrane pemphigoid (MMP) occurring in childhood with exclusively ocular involvement. Case report. A 12-year-old girl presented with mild bilateral conjunctival cicatrization, symblepharon, and peripheral inflammatory keratitis. No other mucosal or cutaneous involvement was present. The findings of an initial conjunctival biopsy with immunofluorescence study were negative for MMP. After a few recurrences of mild corneal and conjunctival inflammation, a second conjunctival biopsy was performed at a different site in the same eye, 30 months later. Only then was the diagnosis of MMP confirmed using positive direct immunofluorescence. The results of a systemic investigation for autoimmune vasculitis were negative. Therapy with dapsone was introduced, and a good response was obtained. MMP is an extremely rare condition in children. This case is the second one reported with exclusively ocular involvement. Despite its rarity in the pediatric age group, MMP should be considered in the differential diagnosis of chronic conjunctival inflammation and fibrosis. Furthermore, a single negative biopsy should not exclude this diagnosis in a suspected clinical case, even in children. When a high degree of suspicion is present, a repeat biopsy may prove useful.

Case of mucous membrane pemphigoid characterized by circulating immunoglobulin A and immunoglobulin G autoantibodies to the gamma‐2 subunit of laminin‐332

Case of mucous membrane pemphigoid characterized by circulating immunoglobulin A and immunoglobulin G autoantibodies to the gamma‐2 subunit of laminin‐332

Three Cases of Mucous Membrane Pemphigoid: Usefulness of Various Local Therapies and Amniotic Membrane Transplantation

Three Cases of Mucous Membrane Pemphigoid: Usefulness of Various Local Therapies and Amniotic Membrane Transplantation

ＶＩＩ型コラーゲンが抗原と思われた粘膜類天疱瘡の１例

82歳，男性。2009年5月より水疱が口腔内に出現し，軟口蓋を中心に出現消退を繰り返し，徐々に疼痛を伴うようになったため，2010年3月当科紹介受診した。病理組織検査では，粘膜上皮下に水疱を認め，正常ヒト皮膚を基質とした蛍光抗体間接法では，抗基底膜IgG抗体が40倍まで陽性であった。1M食塩水で剥離した正常ヒト皮膚を基質とした蛍光抗体間接法では，抗基底膜部抗体は水疱底に反応した。ELISA法による抗デスモグレイン1，3抗体，抗BP180抗体，抗BP230抗体は陰性で，真皮抽出液を用いた免疫ブロットにて患者血清は290kDの蛋白に反応したが，抗VII型コラーゲンELISAは陰性であった。以上の結果より，VII型コラーゲンが抗原と思われる粘膜類天疱瘡と診断した。ミノマイシン，ニコチン酸アミド，コルヒチンは副作用出現のため内服継続できずプレドニゾロン内服にて加療した。

Place of human amniotic membrane immunoblotting in the diagnosis of autoimmune bullous dermatoses

Fine analysis of antiskin autoantibodies can contribute to the differential diagnosis of autoimmune bullous dermatoses. To develop a high-performance immunoblotting method using human amniotic membrane as the antigen source, and to compare it with current laboratory methods. Sera from 113 patients were tested by immunoblotting (IB), rat and monkey oesophagus and salt-split skin indirect immunofluorescence (IIF), and enzyme-linked immunosorbent assay (ELISA) quantification of anti-BP180-NC16a and anti-BP230, or antidesmoglein (Dsg) 1 and 3 antibodies. There were 56 cases of bullous pemphigoid (BP), 22 cases of mucous membrane pemphigoid (MMP), eight cases of epidermolysis bullosa acquisita (EBA), two cases of bullous systemic lupus erythematosus (BSLE), 17 cases of pemphigus vulgaris (PV), and four cases each of pemphigus foliaceus (PF) and paraneoplastic pemphigus (PNP). In BP, the three methods had similar sensitivity (84-89%) for both anti-BP180-NC16a and anti-BP230 antibody detection. In MMP, autoantibodies (mainly directed against BP180 or laminin 332 subunits) were detected in 77% of patients by IB, compared with only 9% by IIF on rat and monkey oesophagus and 36% on salt-split skin, and 14% by anti-BP180-NC16a and anti-BP230 ELISA. In patients with pemphigus, ELISA had 92% sensitivity for anti-Dsg1 and 3, but IB and rat bladder IIF were necessary to confirm PNP by revealing specific and rare patterns (antidesmoplakin I/II, antienvoplakin and antiperiplakin antibodies). IB also revealed anticollagen VII antibodies in 60% of patients with EBA and BSLE, and antibodies to BP180, BP230 and Dsg3 in a few patients who were negative using the other two techniques. Amniotic membrane immunoblotting is an interesting diagnostic tool for bullous diseases, as the entire panel of autoantibodies can be detected with a single extract. This method improves the identification of complex and heterogeneous autoimmune processes in conjunction with IIF and ELISA, and is particularly useful for MMP characterization.

A case of mucous membrane pemphigoid with IgG antibodies against all the α3, β3 and γ2 subunits of laminin-332 and BP180 C-terminal domain, associated with pancreatic cancer

A case of mucous membrane pemphigoid with IgG antibodies against all the α3, β3 and γ2 subunits of laminin-332 and BP180 C-terminal domain, associated with pancreatic cancer

A Case of Mucous Membrane Pemphigoid with Autoantibodies against the Gamma 2 Chain of Laminin 5

A Case of Mucous Membrane Pemphigoid with Autoantibodies against the Gamma 2 Chain of Laminin 5

経過観察中に骨髄異形成症候群を発症した粘膜類天疱瘡の１例

経過観察中に骨髄異形成症候群を発症した粘膜類天疱瘡の１例

粘膜類天ほうそうの２例

Two cases of mucous membrane pemphigoid are presented. In the both cases bullae were formed on the gingivae only, with no involvement of the skin or other mucosa. They were diagnosed as mucous membrane pemphigoid by histopathogical findings and direct immunofluorescence technique. Topical steroids were effective. Both cases healed without scar formation, and there has been no recurrence. These findings suggest the existence of a variant type of mucous membrane pemphigoid that is limited to the oral mucosa and has a good prognosis.

Mucous membrane pemphigoid: Treatment experience at two institutions

The initial oral findings and treatment in 50 cases of mucous membrane pemphigoid are presented. Histologic and immunologic studies were undertaken in each case to confirm the clinical diagnosis. The treatments prescribed are summarized and illustrate that topical steroids are effective, but in some cases systemic steroid therapy with or without other immunologically active drugs is required. A significant number of patients had extraoral manifestations of the disorder.