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In dermatology, lichenoid describes lesions with a violaceous hue that is a clinical reflection of basal cell damage in the epithelium and dense mononuclear infiltrate in the sub-epithelium. The violaceous color results from pigment incontinence due to basal cell damage and the Tyndall effect. Although classically described in lichen planus, a lichenoid hue is noted in the oral mucosa in several other disorders that often lead to diagnostic dilemmas. Early and accurate diagnosis is important for the appropriate management of the underlying condition and prognostication. Dermatologists play a central role in managing such patients since, apart from the oral mucosa findings, the cutaneous features also help to significantly differentiate various conditions. Mimickers of oral lichen planus include nicotine stomatitis, oral submucous fibrosis, oral lichenoid lesions, mucosal discoid lupus erythematosus, pemphigus vulgaris, paraneoplastic pemphigus, mucous membrane pemphigoid, fixed drug eruption, plasma cell cheilitis/gingivitis, and erythema multiforme. While a detailed history and clinical examination can help reach a diagnosis in most cases, histopathology, immunofluorescence, and other relevant investigations help establish the diagnosis., (© 2024 the International Society of Dermatology.)

Background Mucous membrane pemphigoid (MMP) is a rare subepidermal autoimmune blistering disorder. The clinical and demographic parameters of this disease in Indian patients have not yet been elucidated in detail. Objective We aimed to study the clinical and demographic characteristics, disease course, and treatment aspects of MMP patients. Methods The data for this study were obtained by reviewing the case record forms of patients registered in the Autoimmune Bullous Disease (AIBD) Clinic of the Department of Dermatology, Venereology & Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, a tertiary care centre in India. The diagnosis of MMP was established on the basis of clinical and immune-histopathological features which are consistent with standard diagnostic criteria for the disease. Results A total of 52 patients with MMP registered in the AIBD clinic were included. The mean age at disease onset was 50 years and the average age at presentation was 56 years. Females outnumbered males in the study with a ratio of 1.36:1. The oral and ocular mucosae were the most commonly affected sites (82.6% and 63.4% respectively). Visual difficulty was reported by half the patients (26 of 52 patients). IgG, C3, and IgA deposits were detected on direct immunofluorescence (DIF) in 29, 21, and 11 patients, respectively. Serologic analysis was performed in only 7 of the patients and of these, just 1 exhibited a positive result on multivariant ELISA and epidermal pattern of binding on salt split skin indirect immunofluorescence. Most patients were treated with prednisolone (44 of 52). Steroid-sparing adjuvants were used in combination including cyclophosphamide, azathioprine, methotrexate, dapsone, and colchicine. Rituximab was administered in 7 patients with severe or refractory disease. Limitations This is a retrospective analysis of data available from a clinic registry. In patients with negative direct immunofluorescence on biopsy, the diagnosis was based on clinico-pathologic consensus. Conclusion MMP is not as uncommon in India as the paucity of reports suggest. Visual complications are frequent in Indian MMP patients. A high index of suspicion is required for early diagnosis and appropriate treatment to prevent ocular complications.

Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease affecting various mucous membranes, with rare skin involvement. We present a case of a 40-year-old woman with recurring desquamative gingivitis, implicating etanercept, an anti-TNF-α agent, in MMP-like lesions confined to the oral mucosa. Suspicion arose due to temporal correlation between drug administration and lesion onset, confirmed by recurrence upon resumption and resolution upon suspension. Laboratory findings supported MMP diagnosis. Notably, the patient had a history of autoimmune urticaria and axial spondylarthritis. A probable adverse drug reaction was established using the Naranjo scale. Possible mechanisms involve the role of TNF-α in MMP pathophysiology and its interaction with viral triggers, exemplified by severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) infection. This case underscores the intricate relationship among autoimmune conditions, medications, and external factors in mucocutaneous disorders, advocating for comprehensive patient assessment and interdisciplinary collaboration for optimal management. (Oral Surg Oral Med Oral Pathol Oral Radiol YEAR;VOL:page range)., Competing Interests: Declaration of interest None., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) are mucocutaneous autoimmune diseases characterized by blistering lesions of mucous membranes and skin, with very similar clinical manifestations. This study aimed to systematically review the literature on the clinical and demographic profile, diagnostic methods, and treatment of patients with pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP). Studies describing cases of PV and MMP diagnosed by direct immunofluorescence that exhibited intraoral manifestations were included. Thirty-two articles were included, with 18 studies on PV and 15 on MMP, corresponding to 50 and 123 cases diagnosed as PV and MMP, respectively. Most patients with PV (64 %) and MMP (81.3 %) were women in the fifth and sixth decade of life, respectively. The mouth was the primary site of involvement both in PV (71.4 %) and in MMP (91 %). The cheek mucosa and gingiva were the most frequently affected intraoral sites in PV (30 %) and MMP (64.2 %), respectively. Direct immunofluorescence was positive for IgG in all cases of the two conditions. The treatment of choice was systemic corticosteroid therapy for patients with PV (50 %) and topical treatment for patients with MMP (53.7 %). Differences in intraoral site predilection, extraoral involvement, and the results of diagnostic tests allow us to trace the clinical, demographic, and diagnostic profile of PV and MMP that contributes to differential diagnosis and therapeutic management., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Background: Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease (AIBD). Some reports suggest that it has a drug-related pathogenesis especially anti-hypertensive drug., Case Presentation: A 67-year-old man with a 7-year history of essential hypertension was prescribed enalapril maleate for 5 months. He presented at our department with pain, ulcers, and blisters on the oral mucosa. We performed clinical, histopathology, and direct immunofluorescence examinations, and findings were consistent with the diagnostic criteria for MMP. Consequently, we consulted with the cardiovascular physician and agreed to discontinue the enalapril maleate replacing it with irbesartan/hydrochlorothiazide tablets and topical corticosteroid therapies instead. The lesions healed without recurrence., Conclusions: ABID induced by antihypertensive drugs have been reported, and enalapril maleate has been implicated as an antihypertensive agent that may trigger AIBDs, such as MMP. This case highlights the potential relationship between antihypertensive drugs and MMP, of which clinicians should be aware to accurately diagnose and promptly relieve patients' pain., (© 2024. The Author(s).)

Purpose: In order to diagnose mucous membrane pemphigoid (MMP) and pemphigus vulgaris (PV) with gingival expression, clinical data must be compared with immunohistochemical data obtained using direct immunofluorescence (DIF). It is therefore essential to carry out a good quality mucosal biopsy for this vital additional test. To date, no study has been able to effectively guide clinicians in their choice of oral site for biopsy to guarantee the efficient contribution of DIF to diagnosis. We propose a systematic review of the literature and a meta-analysis to clarify this issue., Materials and Methods: Electronic databases and bibliographies of articles were searched in April 2023. The primary outcome was the rate of DIF + contribution to diagnosis according to the location of the oral site biopsied., Results: 16 studies were included. Gingival biopsies showed a rate of DIF + 100% [97%-100%] p = 0.998 I 2  = 0.0% with no heterogeneity for PV, and 90.2% [66.5%-100%] p < 0.001 I 2  = 89.6% with high heterogeneity for MMP. For the other oral sites, this rate was 95.7% [87.4%- 100%] p = 0.011 I 2  = 73.0% with moderate heterogeneity for PV, and 87.4% [70.1%- 98.7%] p < 0.001 I 2  = 92.6% with high heterogeneity for MMP. In addition, meta-regression confirmed the significant association between the appearance of the biopsied mucosa and the rate of DIF + in MMP (p < 0.001), with no influence on residual heterogeneity., Conclusion: The nature of the oral mucosa biopsied does not influence the rate of DIF + to diagnosis. The choice of biopsy site should only take into account the characteristics of the clinical picture and the benefit/risk balance of the surgical protocol. The sample must be taken in healthy aeras as close as possible of active lesions: on the gingiva if the MMP and PV are strictly gingival, on the alveolar mucosa if the whole gingiva is altered and on any healthy mucosa if a large number of oral sites are affected., Clinical Trials: CRD42023392345., (© 2024. The Author(s).)

The occurrence of desquamation, shedding and erythema on marginal and attached gingiva is described as desquamative gingivitis (DG). Various autoimmune/dermatological disorders cause DG.The aim of the present systematic review was to gather information on all possible kinds of treatment for DG, based on specific DG diagnoses.The review was organized following the PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) protocol. An electronic search was conducted in the PubMed, Scopus and Google Scholar databases, up to April 2022. Reviews, letters and studies with less than 2 participants were excluded.Fifteen publications matched the eligibility criteria: 6 case series; 5 clinical trials; 3 randomized clinical trials (RCTs), and 1 cohort study. A total of 330 patients were enrolled, mostly women (81.52%), with an average age of 57.6 years. Diagnostic characteristics corresponded to oral lichen planus (OLP) (n = 249), mucous membrane pemphigoid (MMP) (n = 30), pemphigus vulgaris (PV) (n = 19), plasma cell gingivitis (PCG) (n = 4), erythema multiforme (EM) (n = 1), and non-specified diseases (NSD) (n = 27). Oral lichen planus and MMP were eliminated using oral hygiene instructions with topical clobetasol and/or doxycycline monohydrate. Pemphigus vulgaris, PCG and EM were treated with topical clobetasol.To conclude, each DG case requires personalized treatment, depending on the diagnosis.

Purpose: Analyze the influence of risk factors at presentation in the long-term immunosuppressive therapy (IMT) outcomes of ocular mucous membrane pemphigoid (OMMP)., Design: Retrospective multicenter study., Participants: Patients with OMMP seen at the Duke Eye Center, Tecnologico de Monterrey, and Hospital Clinic of Barcelona from 1990 to 2022., Methods: Data at presentation on demographics, direct immunofluorescence, ocular findings, sites of extraocular manifestations (EOMs), and previous treatments in patients with a clinical or laboratory diagnosis of OMMP, were analyzed with multivariable analysis and Kaplan-Meier plots to identify factors associated with adverse outcomes., Main Outcome Measures: (1) Inflammatory control (no conjunctival inflammation in both eyes at 3 months on IMT); (2) relapse (new-onset inflammation after absolute control in either eye); (3) progression (≥ 1 cicatrizing stage progression in either eye); and (4) vision loss (≥ 2 Snellen lines)., Results: A total of 117 patients (234 eyes), 61% (71/117) of whom were women, with a mean age of 66.6 (SD: 12.4) years (range: 37-97 years) and median follow-up of 34 months (interquartile range: 16-66 months; range: 3-265 months), were enrolled. Inflammatory control was achieved in 57% of patients (67/117), with high-risk EOM (HR-EOM), including esophageal, nasopharyngeal, and/or genital involvement (adjusted odds ratio [aOR]: 12.51; 95% confidence interval [CI]: 2.61-59.99; P = 0.002) and corneal scarring (aOR: 3.06; 95% CI, 1.15-8.14; P = 0.025), as significant risk factors for persistent inflammation. Disease relapse, progression, and vision loss occurred in 20% of patients (23/117), 12% of patients (14/117), and 27% of patients (32/117), respectively. Baseline corneal scarring was a risk factor for relapse (adjusted hazard ratio: 4.14; 95% CI: 1.61-10.62; P = 0.003), progression (aOR: 11.46; 95% CI: 1.78-73.75; P = 0.010), and vision loss (aOR: 3.51; 95% CI: 1.35-9.10; P = 0.010). HR-EOM was associated with stage progression (aOR, 34.57; 95% CI, 6.57-181.89; P<0.001) and vision loss (aOR, 8.42; 95% CI, 2.50-28.42; P = 0.001). No significant differences were found between IMT regimes and relapse (P = 0.169)., Conclusions: Ocular mucous membrane pemphigoid presenting with HR-EOMs and corneal scarring has an increased risk of stage progression and vision loss. Corneal scarring and severe inflammation at baseline were associated with an increased risk of relapse. A disease progression staging system incorporating both the HR-EOMs and corneal involvement is required to predict the visual outcome of OMMP better., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Mucous membrane pemphigoid is a rare autoimmune disease affecting mucosal surfaces. Pediatric cases are exceptionally rare, one subtype being vulvar pemphigoid. Juvenile vulvar pemphigoid can be challenging to diagnose due to its rarity and subtle initial symptoms. We present a case of an 8-year-old girl successfully diagnosed early in the disease course via histopathology, and immunofluorescence. Detecting MMP can be complex due to variations in epitope binding typically not included in commercial ELISA assays, necessitating comprehensive workup. Missed diagnosis may lead to progression to systemic involvement with severe consequences; thus, timely diagnosis and treatment are crucial., (© 2024 The Authors. Pediatric Dermatology published by Wiley Periodicals LLC.)

Purpose: The aim of this study was to report 2 cases of levamisole-adulterated cocaine-induced mucous membrane pemphigoid., Methods: This study is a review of case reports and literature., Results: Two patients presented with bilateral severe purulent conjunctivitis, corneal ulceration, and rapidly progressive forniceal shortening. Both patients were active cocaine users. A complete blood analysis showed a positive antineutrophil cytoplasmic antibody immunofluorescence with a mixed perinuclear antineutrophil cytoplasmic antibody and cytoplasmic-staining antineutrophil cytoplasmic antibody pattern. Direct immunofluorescence examination of conjunctival tissue showed linear deposition of component 3 and immunoglobulins at the basal membrane. A diagnosis of levamisole-adulterated cocaine-induced mucous membrane pemphigoid was made. In case 1, this suspicion was confirmed by investigating remnants of cocaine on the patient's debit card using mass spectrometry, which contained traces of levamisole. In both cases, aggressive immunosuppressive therapy combining systemic corticosteroids and rituximab was able to control the disease. However, by the time these therapies were initiated, significant corneal injury had occurred requiring corneal grafts in both patients., Conclusions: Given the rising abuse of cocaine, it is important that ophthalmologists are made aware of its association with severe atypical cicatricial conjunctivitis. To the best of our knowledge, we present the first case proving the causal relationship between levamisole and ocular cicatricial pemphigoid., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: The purpose of this study is to report ocular cicatricial pemphigoid (OCP) occurring in young patients. Relevant literature is also reviewed., Methods: Medical records of patients aged 30 years or younger who were treated for OCP between August 2021 and May 2023 at a tertiary care eye institute were reviewed. The most common differential diagnoses of cicatrizing conjunctivitis, such as Stevens-Johnson syndrome sequelae, chemical injury, chronic topical/systemic drug use, autoimmune connective tissue disorders, or allergic eye diseases, were ruled out based on clinical history, examination, and the Cicatrizing Conjunctivitis Score described by Shanbhag et al. The diagnosis of OCP was confirmed by positive direct immunofluorescence of oral mucosal and/or conjunctival biopsy in a majority of the patients., Results: Seven patients fulfilled the criteria for a diagnosis of OCP. The mean age at presentation was 21.86 ± 5.25 years (13-28 years). Some of the patients presented with relatively atypical features for OCP such as corneal immune ring infiltrate and bilateral corneal perforation. Six patients exhibited systemic mucosal lesions, and the direct immunofluorescence yield was 85.71%. All patients required aggressive immunosuppressive treatment., Conclusions: OCP is classically described as a rare disease that occurs in old age. This case series highlights the importance of a higher index of suspicion for diagnosing OCP at a younger age. Early administration of immunosuppressive agents can potentially control severe ocular surface inflammation and its sequelae., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Sebaceous carcinoma is a known mimicker of benign conditions, leading to frequent delays in diagnosis and proper treatment. We present two patients with chronic cicatrizing conjunctivitis initially diagnosed as ocular mucous membrane pemphigoid (MMP) and later found to have sebaceous carcinoma. Both patients presented with unilateral conjunctivitis that failed to improve with topical and systemic therapy, eventually developing fornix foreshortening and extensive symblepharon. Case 1 was diagnosed with ocular MMP based on clinical features alone, while Case 2 was diagnosed with biopsy-negative disease. Months to years later, both patients developed lid lesions found to be sebaceous carcinoma and underwent exenteration. As diagnosis and treatment of ocular MMP without positive direct immunofluorescence testing becomes increasingly accepted, clinicians should consider sebaceous carcinoma as the initial diagnosis or as a developing phenomenon during immunosuppression in the setting of chronic inflammation. A low threshold for repeat biopsy should be maintained.

Purpose: The aim of this study was to report a rare observation of corneal superficial plaque formation after topical recombinant human nerve growth factor (rhNGF) treatment for a nonhealing epithelial defect in a patient with advanced mucous membrane pemphigoid, limbal stem cell deficiency, and neurotrophic keratopathy., Methods: This study was a case report., Results: A 72-year-old man with a complex course of mucous membrane pemphigoid, leading to cicatrizing keratoconjunctivits, limbal stem cell deficiency, and neurotrophic keratopathy presented with a recurrent persistent epithelial defect in the right eye. After a long course of unsuccessful epithelial healing, despite various treatment modalities, he was administered topical rhNGF (cenegermin 0.002%; Oxervate, Dompé US Inc., Boston, MA) which successfully resolved the epithelial defect. However, on day 22 posttreatment, an unusual white, thick, adherent corneal superficial plaque formed. rhNGF was stopped and the plaque was carefully removed. Subsequently, there was no recurrence, and the patient's epithelial healing remained stable., Conclusions: Although the successful resolution of the persistent epithelial defect with rhNGF administration was notable, the development of the unusual epithelial overgrowth emphasizes the importance of vigilant monitoring and evaluation when using rhNGF in complex ocular conditions. Making informed decisions on the timing of discontinuing rhNGF can lead to desirable effects of the drug while mitigating additional side effects when managing such challenging cases., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To investigate the long-term outcomes of fornix reconstruction and cicatricial entropion repair in patients with ocular mucous membrane pemphigoid (MMP) and secondary MMP., Methods: Retrospective chart review of patients with MMP undergoing either fornix reconstruction (with amniotic membrane or buccal mucosal graft) or Wies cicatricial entropion repair between January 1, 2000, and September 1, 2020. Patients had a positive mucosal biopsy and (or) clinical features of MMP or secondary MMP. The primary outcome was overall success of fornix reconstruction based on fornix depth maintenance at latest follow-up. Secondary outcomes included resolution of trichiasis, visual acuity, and improvement of subjective symptoms., Results: Eight patients (10 eyes) with a diagnosis of MMP (3 males and 5 females; median age, 71 years) and 4 patients (4 eyes) with a diagnosis of secondary MMP (2 females and 2 male; median age, 87 years) were enrolled. Mean follow-up was 22.7 months (range, 0.3-87.5 months) for MMP patients and 15.4 months (range, 3.0-43.9 months) for secondary MMP patients. For MMP eyes, 30.0% underwent fornix reconstruction, 60.0% underwent entropion repair, and 10.0% received both. Re-formation of symblepharon and loss of fornix depth occurred in all MMP eyes at an average of 6.4 ± 7.0 months postoperatively, and trichiasis recurred in all patients at the last follow-up visit. In secondary MMP patients, 75.0% of the eyes showed recurrence of symblepharon, and 66.7% re-formed trichiasis. Both MMP and secondary MMP patients had short-term symptom improvements., Conclusions: Fornix reconstruction and cicatricial entropion repair in our cohort of MMP and secondary MMP patients resulted in short-term symptomatic improvement, but recurrence was seen, on average, at 6 months postoperatively., (Copyright © 2023 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Objective: The efficiency of B-cell depletion therapy for severe ocular cicatricial pemphigoid (OCP) highlights the key role of B lymphocytes in the immunopathogenesis of OCP. B-cell activating factor (BAFF) is a potent B-cell growth factor and costimulator of immunoglobulin production. Elevated serum BAFF is associated with systemic autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis and bullous pemphigoid. We hypothesize that serum BAFF levels are also increased in patients with OCP., Methods: Sera were collected from 30 patients with new-onset active OCP, 9 with disease in remission, 10 with OCP relapse, and 15 healthy control individuals. An enzyme-linked immunosorbent assay was performed to measure the concentration of serum BAFF., Results: BAFF was significantly higher in patients with new-onset active OCP (700.8 ± 181.8 pg/mL) than in healthy control individuals (564.1 ± 133.2 pg/mL; p = 0.014). No significant difference was found between patients with OCP in remission (585.4 ± 216.2 pg/mL) and healthy control individuals. Patients with disease relapse treated with rituximab had an extremely high concentration of BAFF (1721.9 ± 790.8 pg/mL). Longitudinal analysis of serum BAFF from 6 patients showed that BAFF decreased as the disease went from new onset (895.0 ± 240.8 pg/mL) to remission (625.4 ± 199.8 pg/mL; p = 0.003)., Conclusions: BAFF is involved in the active inflammation of OCP. Targeting BAFF with an antagonist may be therapeutically beneficial for patients with refractory OCP, especially those resistant to rituximab., (Copyright © 2022 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Purpose: The aim of this study was to report a series of 3 patients with ocular graft-versus-host disease (oGVHD) with progressive cicatricial conjunctival changes who were diagnosed with ocular cicatricial pemphigoid (OCP) after conjunctival biopsy., Methods: This study was a retrospective case series., Results: Three patients who received hematopoietic stem cell transplantation for hematologic malignancies developed oGVHD and subsequently were diagnosed with OCP. Case 1 was a 73-year-old woman with oGVHD who developed symblepharon and showed positive IgA, IgG, and C3 staining of the basement membrane zone (BMZ) on conjunctival biopsy, consistent with OCP. She was systemically treated with tacrolimus and prednisone with resolution of conjunctival inflammation. Case 2 was a 68-year-old man with oGVHD who developed symblepharon, severe dry eye, and corneal epithelial defect. An initial conjunctival biopsy was negative, but a repeat biopsy performed 10 years later showed positive BMZ IgA and IgG staining. Healing of the epithelial defect was achieved after treatment with high-dose systemic cyclosporine. Case 3 was a 75-year-old woman with oGVHD who had a nonhealing corneal epithelial defect and symblepharon with positive IgA BMZ staining on conjunctival biopsy, consistent with OCP. The patient responded well to methotrexate with healing of the epithelial defect., Conclusions: Although low-grade conjunctival fibrotic changes may be observed in chronic oGVHD, development of severe and progressive cicatricial changes, including symblepharon formation, should prompt consideration of biopsy to rule out concurrent OCP, the management of which differs from that of oGVHD., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To assess the prevalence of autoimmune diseases (ADs) associated with ocular cicatricial pemphigoid (OCP) and analyze clinical, laboratory, and treatment associations between these entities., Methods: A multicentre cross-sectional study of patients with an OCP diagnosis. The population was divided into two groups according to their association with other ADs or not. Clinical, laboratory and treatment variables were described and compared between groups. A multivariable logistic regression analysis was performed to identify variables that could suggest the association between OCP and ADs., Results: Eighty-eight patients were recruited, with a mean age at diagnosis of 64.3 years (SD 11.9). Biopsy was performed in 86.8% of the patients. There was a median delay of 2 years from the onset of symptoms to diagnosis. Extraocular involvement was evidenced in 11.5%. The group associated with ADs included 24 patients (27.3%). The most prevalent diagnosis was Sjögren´s syndrome. Hypergammaglobulinemia was associated with ADs and OCP, adjusted for age, sex, smoking, skin and mucosal involvement, and erythrocyte sedimentation rate (OR 8.7; 95%CI 1.6-46.8; p = 0.012)., Conclusions: Due to OCP's autoimmune nature, it could coexist with other ADs. This study observed that more than a quarter of the population presented with this association, and hypergammaglobulinemia could suggest it., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Mucous membrane pemphigoid (MMP) is a rare, immune-mediated, vesiculobullous disease that predominantly affects the oral cavity and conjunctiva. In MMP, autoantibodies are directed against hemidesmosomal proteins in the basement membrane zone, most commonly BP180. Clinical signs and symptoms include gingival desquamation, erosions, and ulcerations. Differential diagnoses include other immune-mediated blistering diseases, such as bullous pemphigoid. Definitive diagnosis is reached through history taking, physical examination, tissue biopsy and/or serology testing. MMP, although not curable, is typically managed with topical or systemic corticosteroids, in addition to immunosuppressive therapies and biologic agents in recalcitrant cases. Untreated MMP can lead to life-threatening complications, such as blindness. As a condition that affects the oral cavity, it is important that dentists understand how to recognise, diagnose and manage the disease., (© 2024. The Author(s), under exclusive licence to the British Dental Association.)

Purpose: The aim of this study was to describe the direct immunofluorescence (DIF) findings and factors affecting conjunctival biopsy positivity in patients clinically diagnosed with ocular mucous membrane pemphigoid (OMMP)., Methods: This retrospective observational case series included patients with clinical OMMP who underwent conjunctival biopsy for DIF in at least 1 eye between 2018 and 2021 in an institutional setting. The primary outcome measures were association of age and chronic ocular complications with biopsy positivity., Results: Of 61 patients, DIF positivity was seen in 33 (54.1%) clinically suspected cases of OMMP. Of 39 patients who underwent bilateral biopsy, 23 (59%) were positive, of which 12 (52%) were positive in both eyes while 11 (48%) were positive in 1 eye. Of 22 patients who underwent unilateral biopsy, 10 (45%) were positive. Of the 100 biopsied eyes, 45 (45%) were DIF positive. Among the immunoreactants studied, linear deposition of C3 was seen in all 45 positive eyes (100%). Increasing age was significantly associated with higher likelihood of biopsy negativity ( P = 0.032), whereas a greater Sotozono chronic ocular complication score, indicative of disease severity, was associated with low likelihood of biopsy positivity ( P = 0.0042) and lower overall expression of immunoreactants on DIF ( P = 0.0007)., Conclusions: Older patients and patients with more severe ocular surface disease sequelae are likely to have negative DIF results. To optimize the chances of confirming the diagnosis of OMMP by DIF, both eyes should be biopsied early in the disease course. If 1 eye is being biopsied, the less affected eye must be chosen., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Pemphigus disease and mucous membrane pemphigoid are autoimmune blistering diseases (AIBDs) which may involve both oral and extra-oral tissues. The Bristol Joint Oral Medicine and Dermatology Combined Clinic was set up in 2014, with the primary aim of improving the standard of care for patients with AIBDs. This interdisciplinary approach aimed to address the medical management challenges due to the multisite nature of these AIBDs.We present a narrative report of the clinical work undertaken within this clinic, focused on the management of this patient cohort within a five-year span (2017-2022). This report outlines the multisite nature of AIBDs and the range of topical and systemic treatments that were employed to achieve adequate disease control and optimise outcomes for patients. We reflect on the experiential benefits of this multidisciplinary clinic extended beyond immediate patient benefits to areas such as specialist training, both from a dermatologist's and oral physician's perspective., (© 2024. The Author(s).)

Purpose: To evaluate preferred diagnostic tools and treatment decision-making factors in cases suspicious of mucous membrane pemphigoid (MMP) amongst ophthalmologists and cornea specialists., Methods: Web-based survey, consisting of 14 multiple choice questions, posted to the Cornea Society Listserv Keranet, the Canadian Ophthalmological Society Cornea Listserv, and the Bowman Club Listserv., Results: One hundred and thirty-eight ophthalmologists participated in the survey. Eighty-six percent (86%) of respondents were cornea trained and practiced in either North America or Europe (83%). Most respondents (72%) routinely perform conjunctival biopsies for all suspicious cases of MMP. For those who do not, fear that biopsy will exacerbate inflammation was the most common reason to defer investigation (47%). Seventy-one percent (71%) performed biopsies from perilesional sites. Ninety-seven percent (97%) ask for direct (DIF) studies and 60% for histopathology in formalin. Most do not recommend biopsy at other non-ocular sites (75%), nor do they perform indirect immunofluorescence for serum autoantibodies (68%). Immune-modulatory therapy is started following positive biopsy results for most (66%), albeit most (62%) would not let a negative DIF influence the choice of starting treatment should there be clinical suspicion of MMP. Differences in practice patterns as they relate to level of experience and geographical location are contrasted to the most up-to-date available guidelines., Conclusion: Responses to the survey suggest that there is heterogeneity in certain practice patterns for MMP. Biopsy remains an area of controversy in dictating treatment plans. Identified areas of need should be targeted in future research., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: Ocular mucous membrane pemphigoid (OcMMP) is a rare eye disease characterized by relapsing-remitting or persisting long-lasting inflammatory events associated with progressive scarring. Despite long-term immunomodulating therapy, abnormal fibrosis keeps worsening in patients with OcMMP. This study investigates the fibrotic process in patients with OcMMP, as well as the critical role of the epithelium in modulating the local fibrosis., Methods: In this prospective, observational pilot study, patients affected by long-lasting OcMMP were compared with age- and gender-matched healthy controls. Clinical grading was assessed, and conjunctival biopsy and impression cytology were performed. Conjunctival samples were used for quantifying the expression of transcripts regulating the inflammatory and fibrogenic processes., Results: Ocular surface clinical and functional markers worsened in patients with OcMMP with fibrotic disease progression. In more advanced disease stages, both impression cytologies and conjunctival biopsies revealed increased tissue remodeling and profibrotic markers (α-SMA and TGF-β), and decreased levels of inflammatory markers (I-CAM1, IL-10, and IL-17). Increased epithelial expression of profibrotic markers and histological changes were detected., Conclusions: Chronic OcMMP is characterized by a progressive, aberrant self-sustaining fibrotic process that worsens clinical signs and symptoms. Conjunctival epithelial cells may transdifferentiate into myofibroblast-like phenotypes when chronically exposed to high levels of inflammation, as in the case of OcMMP. Tissue remodeling markers in OcMMP could be used as early diagnostic, prognostic, and therapeutic biomarkers, harvested in a non-invasive and painless procedure such as impression cytologies.

Purpose: The aim of this study was to describe the presenting feature of vortex keratopathy in 3 patients with biopsy-proven ocular mucous membrane pemphigoid (OMMP)., Methods: The first patient was a 52-year-old woman with chronic redness and foreign body sensation for 3 years who presented with unilateral vortex keratopathy. Seven months later, in the same eye, she developed conjunctival signs suggestive of OMMP. The second patient was a 33-year-old woman with similar chronic symptoms that were exacerbated by pterygium surgery. Clinical examination revealed vortex keratopathy in the right eye with subtle conjunctival signs suggestive of OMMP. The third patient was a 70-year-old woman with complaints of repeated episodes of redness and foreign body sensation for 18 months who presented with vortex keratopathy in the right eye and conjunctival signs suggestive of OMMP in the same eye. To confirm the clinical diagnosis, bilateral conjunctival biopsy was performed in all patients., Results: OMMP was diagnosed based on conjunctival signs and confirmed on direct immunofluorescence positivity, demonstrating antibodies characteristic and diagnostic of OMMP in the basement membrane zone. A unique feature that preceded or coexisted with the conjunctival signs was unilateral vortex keratopathy seen in all 3 patients, independent of the stage of the disease at which they presented., Conclusions: Vortex keratopathy can be a presenting feature in patients with OMMP. A complete ocular surface examination, especially checking the medial canthus for keratin and the inferior fornix for foreshortening, is necessary. Conjunctival biopsy should be performed in all cases to confirm the clinical diagnosis wherever required., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: Bullous pemphigoid has previously been linked to radiotherapy, but here we report the first case of MMP suspected to be a consequence of RT., Methods: The patient described is an 85-year-old male who underwent RT to treat squamous cell carcinoma of the palatine tonsil. Shortly after therapy, the patient developed blisters with worsening dyspnea and dysphonia., Results: This patient was successfully treated with a combination of oral immunosuppressants and surgical intervention., Conclusion: This incident underscores that not all episodes of mucosal ulceration following radiation are a result of mucositis and MMP should be considered in the differential., Level of Evidence: Level 4.

Background: Ocular involvement in mucous membrane pemphigoid (MMP) is relatively rare, with a prevalence of 25 cases per million population, equating to approx. 2,100 patients throughout Germany. Diagnosis can be difficult - especially in cases of isolated ocular involvement - and treatment can be complex and lengthy. Immunosuppressants or immunomodulatory drugs are often used. Due to the complexity of diagnosis and treatment, MMP patients are usually referred to specialized centers. The aim of this project was to evaluate the current care situation of patients with ocular MMP in Germany., Methods: A paper-based survey was designed and sent to all university eye clinics and other specialized centers in Germany in April 2020. The survey asked about the existence of a specialized outpatient service, the total annual number of patients with MMP, the annual number of newly diagnosed patients, any interdisciplinary collaboration for diagnostic or therapeutic purposes, as well as the local and systemic therapy used., Results: Of a total of 44 clinics, 28 (64%) responded, reporting a total average of 27 ± 42 (0 - 200) patients and 3.6 ± 2.2 (0 - 10) new cases per year. This corresponds to a total of 741 patients. Only nine (32%) of the responding clinics offer specialized MMP clinics. 93% of the centers collaborate with the local dermatology department. 79% perform serological and histological diagnostics in-house. About half of the centers (n = 16) apply a standardized treatment regime. Systemic glucocorticoids (66.7%) are most commonly used, followed by mycophenolate mofetil and dapsone (57.1%), rituximab (33.3%), azathioprine and cyclophosphamide (28.6%), as well as methotrexate (19.0%). The least frequently used treatment is intravenous immunoglobulin (14.3%)., Conclusion: This survey of German ophthalmology departments obtained data from about one third of the estimated total cohort of all patients with MMP in Germany. These are presumed to be exclusively patients with at least one ocular involvement. The complex care of these patients is usually provided in collaboration with a dermatologist and with the use of systemic anti-inflammatory medication. Currently, an ophthalmological MMP register is being established to better record the epidemiology and care situation of this rare disease in Germany and to improve it in the long term., Competing Interests: Martina Herwig-Carl: Forschungsförderung: EPIC-XS (Projektnr. 823839, gefördert durch das Horizon 2020 Programm der EU), Deutsche Forschungsgemeinschaft (HE5775/5-1); Beratung und Vortragstätigkeit: GlaxoSmithKline. Ich erkläre, dass die anderen Autoren während der letzten 3 Jahre keine wirtschaftlichen oder persönlichen Verbindungen im oben genannten Sinne angeben./ Martina Herwig-Carl: Research grant: EPIC-XS (project no. 823839, funded by the Horizon 2020 programme of the EU), German Research Foundation (HE5775/5-1); lectures and consultancy work: GlaxoSmithKline. I declare that the other authors have not declared any economic or personal connections as defined above in the past 3 years., (Thieme. All rights reserved.)

Pemphigoid diseases are a group of bullous autoimmune diseases characterized by autoantibodies against structural proteins of the dermal-epidermal junction. With a steadily growing aging population, pemphigoid diseases are emerging as a significant medical challenge, because they occur primarily in older individuals. The by far most common disease is bullous pemphigoid, which is clinically characterized by tense blisters, erosions, erythema or urticarial plaques, while severe pruritus is the leading subjective symptom. Mucous membrane pemphigoid predominantly affects surface-close mucous membranes with painful erosions and blisters as well as frequently scarring usually in the mouth, nose, and eyes. Anti-p200 pemphigoid clinically resembles bullous pemphigoid but is much less common. Diagnosis of these diseases involves the combination of clinical evaluation, lesional histopathology, direct immunofluorescence microscopy of a perilesional biopsy and serology. Topical and systemic corticosteroids are the mainstay of pemphigoid diseases treatment. Depending on the severity of the disease, various potentially corticosteroid-sparing therapies, such as dapsone, doxycycline, methotrexate, azathioprine and mycophenolate may be used. In severe courses, treatment with rituximab, cyclophosphamide, intravenous immunoglobulins or immunoadsorption are second- or third-line treatment options. Patients are best managed in centers experience with the management of pemphigoid diseases. Updated national and international guidelines for the diagnosis and treatment of bullous pemphigoid and mucous membrane pemphigoid have recently been published., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

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.

A non-comparative retrospective case-series study was performed on 6 patients (12 eyes) with a confirmed diagnosis of ocular cicatricial pemphigoid (OCP), treated with MicroPulse Transscleral Laser Therapy (MP-TLT), between January 2018 and 2020, with a minimum follow-up of 2 years, in a private ophthalmology practice with offices in Argentina and Colombia. Mean age was 75±6.3 years (66-83 yrs). The severity of ocular cicatricial pemphigoid remained stable 2 years after MP-TLT. Baseline mean IOP was 19.5±5.9 mmHg (13-30 mmHg) and, 24 months after treatment, IOP decreased to a mean of 13.9±1.9 mmHg (10-18 mmHg). The mean IOP reduction was 25.1% ( P : 0.002). The mean number of anti-glaucomatous drugs at baseline was 3.1±0.7, decreasing to 0.6±0.7, 2 years after MP-TLT ( P <0.001). MP-TLT may be considered as a non-incisional approach to glaucoma care for patients with ocular surface diseases, prospective long term studies are needed., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Mucous membrane pemphigoid is an autoimmune disease with variable clinical presentation and multiple autoantigens. To determine whether disease endotypes could be identified on the basis of the pattern of serum reactivity, the clinical and diagnostic information of 70 patients with mucous membrane pemphigoid was collected, and reactivity to dermal or epidermal antigens, using indirect immunofluorescence, and specific reactivity to bullous pemphigoid (BP) autoantigens BP180 and BP230, collagen VII, and laminin 332 were evaluated. Most patients had lesions at multiple mucosae, with the most prevalent being oropharyngeal (mouth, gingiva, pharynx; 98.6%), followed by ocular (38.6%), nasal (32.9%), genital or anal (31.4%), laryngeal (20%), and esophageal (2.9%) sites and skin (45.7%). Autoantigen profiling identified BP180 (71%) as the most common autoantigen, followed by laminin 332 (21.7%), collagen VII (13%), and BP230 IgG (11.6%). Reactivity to dermal antigens predicted a more severe disease characterized by a higher number of total sites involved, especially high-risk sites, and a decreased response to rituximab. In most cases, identification of dermal indirect immunofluorescence reactivity is an accurate predictor of disease course; however, confirmation of laminin 332 reactivity is important, with dermal indirect immunofluorescence positivity because of an increased risk of solid tumors. In addition, the ocular mucosae should be monitored in patients with IgA on direct immunofluorescence., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Mucous membrane pemphigoid refers to a heterogeneous group of autoimmune diseases with subepidermal blister formation that can affect all mucous membranes with varying frequencies. This is a rare disease without any geographic or sexual predisposition that is characterized by recurrent inflammation and progressive scarring. The specific diagnostics can be negative in up to 50% of cases. The diagnosis is predominantly made in patients aged 60-80 years. Ophthalmologists play an important role in the care of affected individuals as the conjunctiva is the second most frequent site of involvement. The treatment is often tedious and primarily consists of long-term systemic immunosuppression., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Objective: The objective of the present study was to explore the clinical characteristics of BP180NC16a autoantibody-positive mucous membrane pemphigoid., Subjects and Methods: Data from 22 patients were collected by retrospective chart review. For the detection of BP180NC16a autoantibody, a commercially available enzyme-linked immunosorbent assay or chemiluminescent enzyme immunoassay kit was used. The patients were classified into the following clinical phenotypes: low-risk patients who had lesions only in the oral mucosa or in the oral mucosa and skin and high-risk patients who had lesions in the oral mucosa and any of the following sites: the eyes, upper respiratory tract, or esophagus., Results: Eleven of the 22 patients (50%) were BP180NC16a-positive at the time of diagnosis. All the positive patients were in the low-risk group. There was a significant difference in the incidence of low-risk mucous membrane pemphigoid between BP180NC16a autoantibody-positive and BP180NC16a autoantibody-negative patients (p = 0.004). Patients' age, disease duration, and oral disease activity scores were not significantly different between the two groups., Conclusion: BP180NC16a reactivity was associated with the clinical phenotype characterized by only oral mucosal lesions or combined oral mucosal and skin lesions. BP180NC16a autoantibody might be useful as a serum marker to predict low-risk mucous membrane pemphigoid., (© 2022 Wiley Periodicals LLC.)

Autoimmune bullous diseases represent a heterogenous group of disorders caused by autoantibodies against adhesion molecules; the location of the target protein determines the level of cleft formation. The spectrum of ocular lesions in autoimmune bullous diseases can range from mild symptoms to severe involvement with sight impairment and even, in some cases, blindness. In pemphigus vulgaris, the prevalence of ocular involvement has been reported to be between 7% and 26%. The most common clinical sign of ocular pemphigus vulgaris is bilateral conjunctivitis with hyperemia. Ocular involvement also occurs in 41% to 70% of patients with paraneoplastic pemphigus. The main ocular manifestations are bilateral cicatrizing conjunctivitis with symblepharon formation, and shortening of the fornices. In mucous membrane pemphigoid, ocular involvement is seen in 61% to 70% of patients; the most frequent ocular finding is cicatricial conjunctivitis. Patients with autoimmune bullous diseases having common ocular involvement should be assessed by an ophthalmologist to avoid serious complications. Diagnostic procedures and treatment require multidisciplinary care based on the close cooperation between dermatologists and ophthalmologists., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023. Published by Elsevier Inc.)

Mucosal pemphigoid is a rare chronic autoimmune disease that also affects the eyes in more than two thirds of all cases. Especially in the early phase of the ocular manifestation, the findings are subtle and the disease is often not recognized. The aim of this article is to provide the clinical aspects of ocular mucosal pemphigoid so that timely diagnostics can be initiated when this disease is suspected., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Purpose: Ocular mucous membrane pemphigoid (OcMMP) is a rare and potentially blinding condition for which consensus treatment guidelines do not exist. The purpose of this study was to assess the effectiveness and safety of various immunomodulatory agents in the treatment of OcMMP in a private practice setting., Methods: We conducted a 10-year retrospective chart review of patients managed with OcMMP (n = 22). The median age at diagnosis was 73 (range: 35-91) years, and 59% (13/22) of patients were female. Visual acuity, Foster stage, and adverse effects (AEs) were documented. Treatment outcomes for each treatment episode were qualified at 3 months as complete response (CR), response (R), or failure (F). After 3 months, CR was then further subqualified as sustained CR, reactivation after initial CR, or AE after initial CR. The Fisher exact test P values were calculated for each outcome in comparison with mycophenolate., Results: Twenty patients were treated with an immunomodulatory agent for a total of 55 treatment episodes. In comparison to dapsone, mycophenolate was more likely to achieve sustained CR (50% vs. 0%, P = 0.022) and R (100% vs. 50%, P = 0.007), and less likely to fail (0% vs. 50%, P = 0.007). Dapsone was also more likely to be discontinued because of AEs than mycophenolate (40% vs. 6%, P = 0.041)., Conclusions: Mycophenolate is a superior first-line agent to dapsone in the treatment of OcMMP. Although not statistically significant, mycophenolate trends toward superiority over methotrexate as well. Mycophenolate is very effective when used in combination with rituximab. Azathioprine remains a reasonable second-line agent., Competing Interests: J. T. Rosenbaum consults for Gilead, AbbVie, Novartis, Roche, Revolo, Corvus, and Horizon. He receives research support from Pfizer and Horizon. He receives royalties from UpToDate. He serves on a data monitoring committee for Celgene-Bristol Myers. The remaining authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

The diagnosis of ocular mucous membrane pemphigoid (MMP) remains a challenge as the timing and choice of diagnostic methods have a decisive influence on its quality. A systematic approach is required that includes a comprehensive medical history, critical evaluation of the clinical findings and targeted laboratory testing. The diagnosis is complicated by the fact that some patients present purely clinically the symptoms of MMP without fulfilling the required immunohistochemical and laboratory criteria. Basically, the diagnosis of ocular MMP is based on three pillars: 1) medical history and clinical findings, 2) positive immunohistological (direct immunofluorescence) tissue sample and 3) specific serological autoantibodies. As the diagnosis of ocular MMP often implies prolonged systemic immunomodulatory treatment in predominantly older patients, the accurate diagnosis and approach are of critical relevance. The aim of this article is to present the recently updated diagnostic procedure., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Overall, conjunctival scarring is rare and often overlooked during routine examination. However, the differential diagnostic distinction of cicatricial mucous membrane pemphigoid from other causes is crucial for further treatment planning. Other entities that lead to conjunctival scarring, are ocular rosacea, ocular graft-versus-host disease, atopic keratoconjunctivitis, infections, trauma and further sometimes very rare diseases. Most of these differential diagnoses have in common that conjunctival scarring does not progress indefinitely if untreated, in contrast to mucous membrane pemphigoid., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Several dermatological conditions may manifest in the oral cavity, particularly those that are immune-mediated, and they must be distinguished from the various other types of oral ulcerations. This chapter discusses the clinical features, pathogenesis, differential diagnosis, and diagnostic features, including histology and immunofluorescence findings, as well as management of vesiculobullous diseases. These diseases include pemphigus Vulgaris, benign mucous membrane pemphigoid, bullous pemphigoid, and epidermolysis bullosa acquisita. These diseases have a significant impact on the quality of life, as they can lead to serious complications, depending on the extent of the disease. Therefore, early recognition is crucial, helping to reduce disease-related morbidity, mortality and prevent life-threatening complications., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Ocular mucous membrane pemphigoid is the manifestation of a systemic autoimmune disease. As autoantibodies circulating in the blood cannot be adequately treated by eye drops, systemic immunosuppressive treatment of this autoimmune disease naturally plays the most important role. Ophthalmic topical or even surgical procedures are only used as supportive measures or usually to control ocular complications that have developed. Patients with the typical clinical picture are treated causally with systemic immunosuppression causally, nurturing eye drops, as well as by minimally invasive surgery if complications arise, if possible in an inflammation-free state, in accordance with the guidelines if the diagnosis is positive but also if the biopsy and serology are repeatedly negative after exclusion of all differential diagnoses. Purely topical anti-inflammatory treatment is insufficient to prevent irreversible progression of scarring conjunctivitis. Corresponding treatment recommendations have been formulated in current European as well as German guidelines and are presented here as an overview., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

The purpose of this study is to report a case of bilateral highly locally invasive conjunctival squamous neoplasia in the clinical setting of ocular cicatricial pemphigoid (OCP), and to review the available literature about this rare association. Case description: A man presented with chronic bilateral conjunctivitis and forniceal foreshortening. He had been previously diagnosed with OCP, and received proper therapy with systemic Dapsone and local steroids. After six months from treatment initiation, the patient complained of worsening symptoms and underwent two more biopsies, revealing a bilateral conjunctival squamous neoplasia. Conclusion: In rare cases, OCP can be associated with conjunctival squamous neoplasia. In case of suspected clinical modifications, it is important to make an early diagnosis by repeating several conjunctival biopsies or by performing less invasive diagnostic techniques (e.g. impression cytology of the ocular surface epithelium) to avoid unnecessary surgical damages.

Mucous membrane pemphigoid (MMP) is a rare disease that presents clinicians with a diagnostic and therapeutic challenge. The aim of this article is to present the German ocular pemphigoid register, which is a retrospective data collection and a collaborative network to improve the care of these patients. It was founded in 2020 and currently comprises 17 eye clinics/cooperation partners. An initial evaluation of the results shows a known epidemiological profile and an expected high proportion of patients with negative diagnostics (48.6%) despite a clinically suspected diagnosis. In this register study predominantly recruiting from eye clinics, the proportion of patients with a strictly ocular involvement was 65.4%. Also of interest was the high number of patients with glaucoma (22.3%) as the most frequent comorbidity. Based on the working group formed, a prospective survey will be conducted in the future, which enables a follow-up., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Objectives: To evaluate the demographic data, ocular and systemic findings, clinical management, and outcomes of patients with ocular cicatricial pemphigoid (OCP)., Materials and Methods: The medical records of 11 patients diagnosed as having OCP in the ophthalmology department of Ege University between 2008 and 2021 were evaluated retrospectively., Results: The patients' mean follow-up time was 14±5.76 months. All eyes (100%) had conjunctival involvement and 18 (81.81%) had corneal involvement. According to the Tauber staging system, 7 (31.81%), 8 (36.36%), and 7 (31.81%) of the eyes were stage 2, 3, and 4, respectively. The diagnosis was confirmed in 6 (66.66%) of 9 patients who underwent biopsy. Amniotic membrane transplantation was performed in 7 eyes, entropion surgery in 2 eyes, and electrocauterization for trichiasis in 5 eyes. Systemic involvement was observed in 45.45% (5/11) of patients, most commonly oral mucosal involvement (18.18%). Review of medical records showed that alkylating agents, steroids, and dapsone were used in patients treated before 2020. Mycophenolate mofetil was preferred to be used in combination with corticosteroids. Although treatment responses before mycophenolate mofetil usage could not be evaluated well because of loss to follow-up, 4 (66.66%) of 6 patients who received steroid treatment combined with mycophenolate mofetil showed partial or complete clinical remission. No serious side effects and drug withdrawal were observed., Conclusion: OCP is a sight-threatening autoimmune disease that affects older adults. Although positive biopsy results are valuable for diagnosis, negative results do not exclude the diagnosis. The main treatment is systemic immunosuppressives. Disease activity can be suppressed, especially with early initiation of drug therapy. These patients require a multidisciplinary approach. Especially in the presence of isolated ocular findings, ophthalmologists should be able to make the decision to start immunosuppressive treatment, and systemic treatment should not be delayed., Competing Interests: Conflict of Interest: No conflict of interest was declared by the authors., (©Copyright 2023 by Turkish Ophthalmological Association | Turkish Journal of Ophthalmology, published by Galenos Publishing House.)