Clinical Characteristics of Ocular Mucous Membrane Pemphigoid: A Systematic Review and Meta-Analysis.

PURPOSE: To synthesize the evidence and generate a combined weighted measure on the frequency of ocular manifestations of mucous membrane pemphigoid (OMMP). METHODS: Systematic literature review and meta-analysis, searching PubMed, Embase, VHL, and Google Scholar. Articles reporting patients with mucous membrane pemphigoid and ocular involvement were included. At least, two reviewers independently and in parallel participated in all the following phases; preliminary screening, full-text review, risk of bias assessment by validated tools, and data extraction. Qualitative analysis and meta-analysis were conducted. This study was previously registered in PROSPERO (CRD42023451844). RESULTS: Thirty-five studies met the inclusion criteria, comprising 1,439 patients and 1,040 eyes summarized in qualitative analysis. Twenty-eight studies were included in the meta-analysis. Ages included ranged from 60.4 to 75 years. Women were reported with more frequency. The mean time for diagnosis was 55.1 months, usually with bilateral ocular disease in 90% (95% CI 78%; 96%). Trichiasis and entropion were the most frequent manifestations in up to 92%, followed by symblepharon and punctate keratitis. Ankyloblepharon, persistent epithelial defects, and visual impairment were less frequent complications. Direct immunofluorescence positivity in conjunctival biopsies was 54% (95% CI 43%; 64%). Extraocular involvement was highly frequent, being oral and skin involvement the most frequently reported. CONCLUSIONS: Our systematic review and meta-analysis evidenced that patients around 60 years of age are the most affected population with a female preponderance, usually with bilateral ocular involvement. Trichiasis and entropion were the most frequent findings; although visual impairment and persistent epithelial defects were less reported, they should not be overlooked in suspected OMMP.

Pemphigus vulgaris versus Mucous Membrane Pemphigoid: Case Series

Pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) are bullous autoimmune diseases that reach the oral mucosa and have common clinical features. The objective of the study was to present and compare the clinical manifestations of PV and MMP and the results of applied treatments. A case series of a stomatology service from 1985 to 2018. Data collection included epidemiological data, comorbidities, medications in use, duration of symptoms before the first visit, previous treatment, symptomatology, clinical description of lesions, presumptive diagnosis, histopathological description, extraoral manifestations, final diagnosis, treatment and follow-up. The medical records of 25 patients were analysed, 19 of whom were diagnosed with MMP and 6 with PV. The female gender was prevalent in MMP (84 %) and the male gender in PV (67 %). More than 60 % of patients complained of pain at their first visit. Patients with MMP took on average 6 months to seek professional help and patients with PV, about 2 months. Desquamative gingivitis was the most common lesion (63 %) in MMP and non-gingival ulcers (67 %) in PV. Minimal therapy was effective in all cases of MMP, and in PV one individual required minimal adjuvant therapy due to worsening of the case. Patients with PV have more intense signs and oral symptoms and may need more intensive treatment than patients with MMP. The use of topical and/or systemic corticosteroids was sufficient for most cases in both diseases.

Pénfigo vulgar (PV) y Penfigoide de la Membrana Mucosa (PMM) son enfermadades autoinmunes ampollosas que llegan a la mucosa oral y tienen características clínicas comunes. El objetivo de este estudio fue presentar y comparar las manifestaciones clínicas de PV y PMM y los resultados de los tratamientos aplicados. En el análisis se incluyó una serie de casos de un servicio de estomatología de 1985 a 2018. La recolección de información incluyó datos epidemiológicos, comorbilidades, medicamentos en uso, duración de los síntomas antes de la primera visita, tratamientos previos, sintomatología, descripción clínica de las lesiones, diagnóstico presuntivo, descripción histopatológica, manifestaciones extraorales, diagnóstico final, tratamiento y seguimiento. Se analizaron las historias clínicas de 25 pacientes, 19 de los cuales fueron diagnosticados de PMM y 6 de PV. El sexo feminino fue prevalente en PMM (84 %) y el sexo masculino en PV (67 %). Más del 60 % de los pacientes se quejaron de dolor durante la primera consulta. Los pacientes con PMM tardaron en promedio 6 meses en buscar ayuda profesional y los pacientes con PV, alrededor de 2 meses. La gingivitis descamativa fue la lesion más común (63 %) en PMM y las úlceras non gengivales (67 %) en PV. La terapia mínima fue efectiva en todos los casos de PMM, y en PV un individuo requirió terapia adyuvante mínima debido al empeoramiento del caso. Los pacientes con PV tienen signos y síntomas orales más intensos y pueden necesitar un tratamiento más intensivo que los pacientes con PMM. El uso de corticosteroides tópicos y/o sistémicos fue suficiente para la mayoría de los casos en ambas enfermedades.

Dermatologic manifestations of multisystem inflammatory syndrome in children during the COVID-19 pandemic

Abstract Objectives: multi-system ınflammatory syndrome in children (MIS-C) is an immune-mediated process that develops after infections like SARS-CoV-2. The authors aimed to reveal the mucocutaneous findings of patients diagnosed with MIS-C at presentation and evaluate the frequency of these mucocutaneous findings and their possible relationship with the severity of the disease. Methods: A prospective study was conducted of 43 children admitted to a tertiary hospitals between January 2021 and January 2022 who met Centers for Disease Control and Prevention criteria for MIS-C. Results: 43 children (25 [58.1%] male); median age, 7.5 years [range 0.5-15 years]) met the criteria for MIS-C. The most common symptom was cutaneous rash 81.4%, followed by gastrointestinal symptoms 67.4%, oral mucosal changes 65.1%, and conjunctival hyperemia 58.1%. The most common mucosal finding was fissured lips at 27.9%, diffuse hyperemia of the oral mucosa at 18.6%, and strawberry tongue at 13.9%. Urticaria (48.8%) was the most common type of cutaneous rash in the present study's patients. The most common rash initiation sites were the trunk (32.6%) and the palmoplantar region (20.9%). The presence or absence of mucocutaneous findings was not significantly associated with disease severity. Study limitations: The number of patients in the this study was small. Conclusions: The present study's prospective analysis detected mucocutaneous symptoms in almost 9 out of 10 patients in children diagnosed with MIS-C. Due to the prospective character of the present research, the authors think that the characteristic features of cutaneous and mucosal lesions the authors obtained will contribute to the literature on the diagnosis and prognosis of MIS-C.

Skin, mucosa and nail findings in hospitalized pediatric patients with Coronavirus disease-2019 (COVID-19)

Abstract Background: Cutaneous manifestations of Coronavirus Disease-2019 (COVID-19) disease have not yet been fully described in hospitalized pediatric patients. Objectives: This prospective study aims to demonstrate the skin, mucosal, and nail findings of hospitalized children with COVID-19. Methods: The authors included hospitalized pediatric patients. Two dermatologists examined skin, hair, nails, and mucosa. Patients with drug eruptions were excluded with an anamnesis, clinical and laboratory test results. Results: Out of 46 enlisted patients, 19 (41,3%) patients displayed skin, mucosal or nail findings. Skin findings were seen on 14 (30.4%) patients. Ten (22%) patients presented skin findings matching described patterns. Half of the patients with patterned rashes had confluent erythematous/maculopapular/morbilliform rashes. Eleven out of 46 (23.9%) patients developed periorbital erythema and edema. Ten (22%) patients had at least one oral mucosal finding. One telogen effluvium, one blue nail, and one flag sign on nails were noticed. Nine (19.5%) patients out of 46 had developed MIS-C. MIS-C patients had mucocutaneous manifestations except one (88.8%). Study limitations: The authors have detected a higher rate of mucocutaneous manifestations compared to out-patients with mild COVID-19 because the study is based on hospitalized patients only. Conclusions: Pediatric COVID-19 patients are more susceptible to developing mucocutaneous manifestations compared to adults. The authors propose COVID-19 should be acknowledged as one of the viral exanthem rashes of childhood. The authors noticed that the most common findings were periorbital erythema and edema. The confluent erythematous/maculopapular/morbilliform rashes appear to be the most common patterns associated with severe COVID-19.

Oral lesions after infection by SARS CoV 2: two case reports / Lesiones bucales posteriores a infección por SARS CoV 2 presentación de dos casos

Introduction: The SARS CoV 2 infection has resulted in several health, economic, and social crises in all areas. The disease shows a substantial biological diversity in humans causing a series of sequels in the trans- or post-infection period in the entire organism. Case Report: The manifestations that occur in the oral cavity and pharynx have not been evaluated. In this study, two clinical cases are reported. The first patient, a 67-year-old male, presents erosive lesions on the dorsal surface of his tongue after SARS CoV 2 infection. Results: Therapy consisting of reinforcing oral cleaning, use of antifungal solutions, mouthwashes containing superoxidation solution and B complex was given to the patient. The reported lesions improved satisfactorily. The second case, a 47-year-old male patient, presented vesiculobullous lesions on the lingual and labial mucosa accompanied by severe painful symptoms after SARS CoV 2 infection. An incisional biopsy was performed. The histopathological result was compatible with pemphigus vulgaris, and the treatment protocol was started with 0.1% topical mometasone and 2g miconazole gel, observing adequate involution of the lesions after 20 days. Conclusions: The aim of this study is to report on the lesions affecting the oral cavity and pharynx in post-COVID patients with the aim of carrying out a thorough intraoral examination, establishing a clinical or histopathological diagnosis to implement a specific treatment plan in each case to improve the health and quality of life of the patients. Keywords: SARS-CoV-2; Oral manifestations; Oral ulcer; Pemphigus; Mouth; Mucous membrane.

Introducción: La infección por virus de SARS CoV 2 ha dejado a su paso una estela de crisis en materia de salud, económica, social y en todos los ámbitos a la fecha seguimos realizando la observación del comportamiento de la enfermedad en los seres humanos con una diversidad biológica importante y que ha traído como consecuencia una serie de secuelas que se presentan en el periodo trans o posterior a la infección en toda la economía corporal. Reporte de Caso: Se ha evaluado poco las manifestaciones que se presentan en la cavidad bucal y faringe; se presentan dos casos clínicos el primero paciente masculino de 67 años de edad posterior a la infección por SARS CoV 2 presenta diluciones de continuidad en bordes laterales de la lengua se indica terapia y refuerza limpieza bucal, antimicótico, colutorios con solución de superoxidación y complejo B, las úlceras involucionan de manera satisfactoria. Resultados: El segundo caso masculino de 47 años posterior a la infección por SARS CoV 2 debuta con lesiones vesículo-ampollosas en mucosa lingual, labial con sintomatología dolorosa severa, se realiza biopsia incisional donde el resultado histopatológico es compatible con pénfigo vulgar, se inicia protocolo de tratamiento con mometasona tópica al 0.1% y miconazol gel 2g observándose una adecuada involución de las lesiones a los 20 días. Conclusiones: El objetivo de este trabajo es poner en contexto de la comunidad médica y científica las lesiones concernientes a la cavidad bucal y faringe que están presentando los pacientes postcovid con el objetivo de realizar una exhaustiva exploración intraoral, establecer un diagnóstico clínico o histopatológico y con base en esto instaurar un plan de tratamiento específico en cada caso en particular con el fin fundamental de mejorar la salud y calidad de vida del paciente.

Laryngeal Mucous Membrane Pemphigoid as an Immune-related Adverse Effect of Pembrolizumab Treatment.

Pembrolizumab is an immune checkpoint inhibitor (ICI) approved for multiple indications in a variety of malignancies. Although generally well tolerated, the potential for significant adverse effects, specifically immune related adverse effects (irAEs) needs to be taken into consideration. Several cases of bullous pemphigoid have been reported as a cutaneous adverse effect of ICIs since 2015, and there are recent reports of mucous membrane pemphigoid (MMP). We present the case of an 84-year-old male with metastatic urothelial carcinoma on treatment with pembrolizumab, who developed laryngeal mucous membrane pemphigoid as an irAE. The diagnosis was based on patient's clinical history and serologic testing, and supported by symptomatic improvement after ICI discontinuation and immunosuppression. Pembrolizumab-induced MMP is a newly described and infrequent irAE, requiring early suspicion and close monitoring for its diagnosis and management.

Dermatologic manifestations of multisystem inflammatory syndrome in children during the COVID-19 pandemic.

OBJECTIVES: multi-system inflammatory syndrome in children (MIS-C) is an immune-mediated process that develops after infections like SARS-CoV-2. The authors aimed to reveal the mucocutaneous findings of patients diagnosed with MIS-C at presentation and evaluate the frequency of these mucocutaneous findings and their possible relationship with the severity of the disease. METHODS: A prospective study was conducted of 43 children admitted to a tertiary hospitals between January 2021 and January 2022 who met Centers for Disease Control and Prevention criteria for MIS-C. RESULTS: 43 children (25 [58.1%] male); median age, 7.5 years [range 0.5‒15 years]) met the criteria for MIS-C. The most common symptom was cutaneous rash 81.4%, followed by gastrointestinal symptoms 67.4%, oral mucosal changes 65.1%, and conjunctival hyperemia 58.1%. The most common mucosal finding was fissured lips at 27.9%, diffuse hyperemia of the oral mucosa at 18.6%, and strawberry tongue at 13.9%. Urticaria (48.8%) was the most common type of cutaneous rash in the present study's patients. The most common rash initiation sites were the trunk (32.6%) and the palmoplantar region (20.9%). The presence or absence of mucocutaneous findings was not significantly associated with disease severity. STUDY LIMITATIONS: The number of patients in the this study was small. CONCLUSIONS: The present study's prospective analysis detected mucocutaneous symptoms in almost 9 out of 10 patients in children diagnosed with MIS-C. Due to the prospective character of the present research, the authors think that the characteristic features of cutaneous and mucosal lesions the authors obtained will contribute to the literature on the diagnosis and prognosis of MIS-C.

A comparative study of conventional intermarginal split lamella with labial mucous membrane graft versus sutureless procedure using fibrin sealant to manage major trichiasis.

OBJECTIVE: In the intermarginal split lamella with labial mucous membrane graft procedure to manage major trichiasis, the graft is usually sutured in the receptor bed using 6-0 polyglactin sutures. We aimed to compare the use of fibrin sealant to seal the graft to the receptor bed versus the conventional technique using sutures. METHODS: This is a retrospective comparative study of patients who underwent conventional intermarginal split lamella with labial mucous membrane graft or sutureless procedure using fibrin sealant (Tisseel, Baxter Healthcare Corp) between 2016 and 2021. Etiology of the trichiasis, procedure duration, postoperative discomfort and edema, complications, and follow-up period were extracted from these patients' charts. RESULTS: Twenty-seven eyelids from 19 patients underwent the procedure: twelve patients underwent the sutureless procedure, while seven underwent the conventional procedure. Mean follow-up was 8.4 ± 2.9 months and 13.7 ± 6.5 months for the sutureless and conventional groups, respectively. Patients who underwent the sutureless procedure reported no postoperative foreign body sensation, while 71.4% of patients who underwent the conventional procedure reported some degree of ocular discomfort. In the sutureless group, operating time and postoperative edema were significantly reduced. Labial mucous membrane graft dehiscence was observed in one eyelid (8.3%) on the first postoperative day in the sutureless group. No dehiscence was observed in the conventional technique group. CONCLUSION: The use of fibrin sealant showed to be a good alternative to conventional absorbable sutures. Advantages include expedited operating time, decreased postoperative discomfort, and expedite postoperative recovery.

Skin, mucosa and nail findings in hospitalized pediatric patients with Coronavirus disease-2019 (COVID-19).

BACKGROUND: Cutaneous manifestations of Coronavirus Disease-2019 (COVID-19) disease have not yet been fully described in hospitalized pediatric patients. OBJECTIVES: This prospective study aims to demonstrate the skin, mucosal, and nail findings of hospitalized children with COVID-19. METHODS: The authors included hospitalized pediatric patients. Two dermatologists examined skin, hair, nails, and mucosa. Patients with drug eruptions were excluded with an anamnesis, clinical and laboratory test results. RESULTS: Out of 46 enlisted patients, 19 (41,3%) patients displayed skin, mucosal or nail findings. Skin findings were seen on 14 (30.4%) patients. Ten (22%) patients presented skin findings matching described patterns. Half of the patients with patterned rashes had confluent erythematous/maculopapular/morbilliform rashes. Eleven out of 46 (23.9%) patients developed periorbital erythema and edema. Ten (22%) patients had at least one oral mucosal finding. One telogen effluvium, one blue nail, and one flag sign on nails were noticed. Nine (19.5%) patients out of 46 had developed MIS-C. MIS-C patients had mucocutaneous manifestations except one (88.8%). STUDY LIMITATIONS: The authors have detected a higher rate of mucocutaneous manifestations compared to out-patients with mild COVID-19 because the study is based on hospitalized patients only. CONCLUSIONS: Pediatric COVID-19 patients are more susceptible to developing mucocutaneous manifestations compared to adults. The authors propose COVID-19 should be acknowledged as one of the viral exanthem rashes of childhood. The authors noticed that the most common findings were periorbital erythema and edema. The confluent erythematous/maculopapular/morbilliform rashes appear to be the most common patterns associated with severe COVID-19.

Tratamiento del penfigoide de membranas mucosas con ácido hialurónico en gel al 0,2%. Reporte de caso / Treatment of mucous membrane pemphigoid with 0.2% hyaluronic acid gel. Case Report

RESUMEN El Penfigoide de membranas mucosas es un trastorno ampollar subepitelial, inflamatorio, autoinmune que afecta las mucosas del cuerpo. El 80% de los casos se presentan en la mucosa bucal. El tratamiento consiste en la administración de corticoesteroides y antimicrobianos. El objetivo de este articulo es describir la eficacia del ácido hialurónico en gel al 0,2% en el tratamiento del penfigoide de membranas mucosas. Se reporta el caso de una mujer de 57 años de edad con artritis reumatoide y penfigoide de membranas mucosas en el labio superior e inferior con tres meses de evolución. Las lesiones son tratadas con ácido hialurónico en gel al 0,2% obteniendo excelentes resultados. El tratamiento con ácido hialurónico demostró efectividad en la reducción del dolor y inflamación, sanando las lesiones sin necesidad de suspender o ajustar la dosis de los fármacos.

ABSTRACT Mucous membrane pemphigoid is a subepithelial, inflammatory, autoimmune blistering disorder that affects the body's mucous membranes. Most of the cases appear in the oral mucosa. Corticosteroids and antimicrobials are the election treatment. This article describes the efficacy of 0.2% hyaluronic acid gel in mucous membrane pemphigoid. We report a case of a 57-year-old female patient with rheumatoid arthritis and mucous membrane pemphigoid in the upper and lower lips with three months of evolution. Lesions were treated with 0.2% hyaluronic acid gel, obtaining excellent results. The treatment of hyaluronic acid to mucous membrane pemphigoid demonstrated effectiveness in reducing inflammation, pain, and healing of the mucosa, without the need to suspend or adjust the dose of the drugs.

Assessment of dynamic thiol-disulfide homeostasis in patients with lipoid proteinosis (Urbach-Wiethe syndrome)

SUMMARY OBJECTIVE: Lipoid proteinosis is a rare autosomal recessive genetic dermatological disease that occurs due to the accumulation of hyaline material in the skin and mucous membranes. This study aimed to investigate whether dynamic thiol-disulfide homeostasis is a new marker of oxidative stress in patients suffering from lipoid proteinosis. METHODS: The study group involved 17 patients with lipoid proteinosis and 17 healthy controls with same gender and age. Native thiol, total thiol, disulfide levels, and thiol-disulfide indexes were measured with the fully automated spectrophotometric method described by Erel and Neselioglu, and the results of the two groups were statistically analyzed. RESULTS: Serum total thiol and native thiol levels were significantly lower in lipoid proteinosis group compared to the control group (p=0.020 and p=0.014, respectively). The disulfide levels were found to be higher in lipoid proteinosis group, but there was no significant difference between two groups. CONCLUSIONS: Impaired dynamic thiol-disulfide homeostasis was observed in lipoid proteinosis patients, suggesting that thiol-disulfide homeostasis may have a role in the pathogenesis of this disease.

The evolution of the modified osteo-odonto-keratoprosthesis, its reliability, and long-term visual rehabilitation prognosis: An analytical review.

An exhaustive search of the world's literature was performed to analyze all case reports and series on the modified osteo-odonto-keratoprosthesis (MOOKP) published up to January 2022. The demographic profile, the primary indication for surgery, surgical technique variations, postoperative medical management, long-term functional and anatomical outcomes, and intra- and postoperative complications were analyzed and compared. Additionally, some of the authors' (GI, VP, and GA) unpublished MOOKP cases were studied. An extensive literature search yielded 37 case series and case reports. Overall, 958 patients were analyzed. The most common indication for surgery was autoimmune disease (39.1%), closely followed by chemical injury (38.8%). The most common intraoperative complications (21.67%) included maxillofacial, vitreous hemorrhage/vitritis, and mucosal. The most common postoperative complications (78.4%) were lamina and oral mucosa-associated, secondary glaucoma, and choroid/retinal detachment. Follow-up periods ranged from one to 364 months (median: 36.7 months). Altogether, 78% of patients achieved a visual acuity of 20/400 or better at the end of the follow-up period, and 91.2% improved at least temporarily after MOOKP surgery. Mean anatomic success at the end-of-follow-up for all patients was 88.25% (range, 50-100%). The long-term anatomic and functional success of the MOOKP makes it a reliable option for visual rehabilitation in patients with bilateral corneal blindness and end-stage ocular surface disease. This review aims to describe the evolution of the MOOKP procedure, analyzing all published case series for its long-term reliability, visual and anatomical outcomes, complications, and future directions.

Ocular cicatricial pemphigoid. From a rheumatology and ophthalmology point of view / Penfigoide ocular cicatrizal, enfoque diagnóstico y terapéutico integral entre el oftalmólogo y el reumatólogo

ABSTRACT Ocular cicatricial pemphigoid (OCP) is a chronic, immune-mediated, bullous, cicatricial disease within the spectrum of mucocutaneous membranous pemphigoids (MMP). Although the diagnosis is often ophthalmological, due to the autoimmune nature of the pathology, it requires a joint approach with rheumatologists and immunologists. The objective of this narrative review was to explore the evidence available in the literature from 2000 to 2020 with respect to clinical manifestations, diagnosis, and treatment. The clinical presentation varies widely, from mild cases with slow progression of years of progression, to severe cases with a torpid and rapidly progressive evolution to fibrosis, refractory to multiple treatments. A com plete evaluation of the patient will help guide the diagnosis. The gold standard for diagnosis is conjunctival biopsy with direct immunofluorescence, although on occasions it can be reached if the symptoms are characteristic. Treatment is local and systemic according to its severity and evolution. The evidence on topical and systemic therapeutics is obtained mainly from uncontrolled observational and experimental studies. Immunomodulatory therapy has made it possible to preserve vision and, in many cases, prevent sequelae. The evolu tion is linked to the early diagnosis and immunosuppressive treatment, so it is essential to be aware of this disease, the diagnostic methods, as well as the immunomodulating and immunosuppressive therapies available.

RESUMEN El penfigoide ocular cicatrizal (POC) es una enfermedad crónica, inmunomediada ampollar, mucosinequiante, comprendida dentro del espectro de penfigoides membranosos mucocutáneos (PMM). El diagnóstico es, con frecuencia, oftalmológico, pero debido al carácter autoinmune de la patología, requiere el abordaje en conjunto con reumatólogos e inmunólogos. El objetivo de esta revisión narrativa fue explorar la evidencia disponible en la literatura, desde el año 2000 hasta el 2020, en lo que respecta a sus manifestaciones clínicas, diagnóstico y tratamiento. La presentación clínica varía ampliamente, desde casos leves con progresión lenta de años de evolución hasta casos severos con evolución tórpida y rápidamente progresiva a la fibrosis, refractarios a múltiples tratamientos. Una evaluación completa del paciente ayudará a guiar el diagnóstico. El estándar de oro diagnóstico es la biopsia conjuntival con inmunofluorescencia directa, si bien en ocasiones puede diagnosticarse por la clínica característica. El tratamiento es local y sistêmico de acuerdo con su severidad y evolución. En los últimos 20 anos, la evidencia sobre los tratamientos tópicos y sistêmicos corresponde en su mayoría a estudios observacionales y experimentales no controlados. Los métodos de tratamiento inmunomoduladores han permitido preservar la visión y, en muchos casos, prevenir secuelas. La evolución está ligada al diagnóstico temprano y a los tratamientos disponibles, por lo que es fundamental el conocimiento de esta patología, los métodos diagnósticos y los tratamientos inmunomoduladores e inmunosupresores.

Photobiomodulation of gingival lesions resulting from autoimmune diseases: systematic review and meta-analysis.

OBJECTIVES: To evaluate the effects of photobiomodulation (PBM) in gingival lesions resulting from autoimmune diseases; to compare PBM and topical corticosteroid (CS) treatment; and to assess PBM outcome over time of follow-up. MATERIALS AND METHODS: A comprehensive electronic search was performed in four electronic databases. Treatment effects were measured through visual analog scale of pain (VAS) and clinical evolution of lesion (Thongprasom scale for oral lichen planus (OLP)). Meta-analysis was performed to compare PBM with topical corticosteroid treatment and to evaluate PBM effect over time of follow-up. RESULTS: Seventeen studies were included in this review, of which six were used for the meta-analysis. Meta-analysis results showed no significant differences between PBM and topical CS in pain reduction at baseline (MD = 0.20, 95% CI = - 0.92, 1.32, p = 0.72) and 60-day follow-up (MD = 0.63, 95% CI = - 3.93, 5.19, p = 0.79); however, VAS showed significant pain reduction when compared before and after PBM at 30-day (MD = - 3.52, 95% CI = - 5.40, - 1.64, p = 0.0002) and 60-day (MD = - 5.04, 95% CI = - 5.86, - 4.22, p < 0.00001) follow-up. Thongprasom clinical scale for OLP also showed significant improvement at 30-day follow-up (MD = - 2.50, 95% CI = - 2.92, - 2.08, p < 0.00001) after PBM. CONCLUSION: PBM led to significant reduction of pain and clinical scores of the lesions, not having shown significant differences when compared to topical CS. CLINICAL RELEVANCE: PBM has been used in the treatment of autoimmune gingival lesions, but so far there is little strong evidence to support its use.

Hyaluronic Acid Concentration in Female Vocal Folds With Reinke's Edema.

OBJECTIVE: The aim of the present study was to investigate hyaluronic acid (HA) concentrations in vocal folds among patients with Reinke's edema. STUDY DESIGN: Prospective and experimental study. SETTING: Single tertiary center. METHODS: An HA binding protein isolated from bovine nasal cartilage was used to identify and isolate the HA from samples. Plates coated with biotin-conjugated binding protein and streptavidin-europium conjugate were sequentially incubated with 18 Reinke's edema samples and 11 female vocal fold cover samples from cadavers (the superficial layer of the lamina propria; control group). After the release of europium from streptavidin in enhancement solution, final fluorescence was measured in a fluorometer. RESULTS: The mean HA concentration in Reinke's edema vocal folds was significantly higher than that in the control vocal folds (9.2 × 103 vs 0.9 × 103µg/g). CONCLUSION: Vocal fold covers affected by Reinke's edema present a higher concentration of HA than do vocal fold covers with no edema.

Alternative Oral Mucous Membrane Pemphigoid Therapy Using Tacrolimus: A Case Report

ABSTRACT: Mucous membrane pemphigoid (MMP) is a blistering disease that predominantly involves the mucous membranes and that can lead to major negative impacts on patient quality of life. The first-line MMP treatment is based on the use of topical and systemic corticosteroids. In this report, we presented a 45-year-old female patient presented blisters in the inferior gingiva for over 8-months. The patient reported being allergic to corticosteroids. Under the clinical hypothesi s of oral lichen planus and MMP, an incisional biopsy was performed, and the histopathological diagnosis of MMP was established. Thus, it was instituted an alternative therapy with tacrolimus 0.03 %. The patient showed an excellent clinical outcome with no recurrence five months after the end of therapy. Tacrolimus 0.03 % may represent an effective therapeutic alternative in MMP treatment and may be used in cases of hypersensitivity to standard therapy.

RESUMEN: El penfigoide de la membrana mucosa (PMM) es una enfermedad ampollosa que afecta predominantemente a las membranas mucosas y que puede provocar importantes impactos negativos en la calidad de vida del paciente. El tratamiento de primera línea de PMM se basa en el uso de corticosteroides tópicos y sistémicos. En este informe, presentamos un caso de una paciente femenina de 45 años que presentó ampollas en la encía inferior durante más de 8 meses. La paciente informó ser alérgica a los corticosteroides. Bajo la hipótesis clínica de liquen plano oral y PMM, se realizó una biopsia incisional y se estableció el diagnóstico histopatológico de PMM. Por lo tanto, se instituyó una terapia alternativa con tacrolimus tópico al 0,03 %. La paciente mostró un excelente resultado clínico sin recurrencia después de 5 meses de la terapia final. Tacrolimus 0,03 % puede representar una alternativa terapéutica efectiva en el tratamiento de PMM y se puede usar en casos de hipersensibilidad a la terapia estándar.

Penfigoide de las mucosas a lo largo de los años en nuestro Servicio / Mucous membrane pemphigoid through the years in our Department

Introducción: el penfigoide de las mucosas (PM), antes llamado mucoso, cicatrizal o mucosinequiante, representa un grupo heterogéneo de enfermedades ampollares autoinmunes inflamatorias crónicas que comprometen las mucosas o la piel, con tendencia a dejar secuelas cicatrizales. Existen autoanticuerpos contra distintos componentes de la zona de la membrana basal (BPAG1, BPAG2, integrina α6ß4, laminina 332, colágeno VII, entre otros), por lo que la inmunofluorescencia directa (IFD) es de suma importancia, así como la clínica, para su diagnóstico.Objetivo: realizar una revisión de los casos de PM diagnosticados durante un período de 24 años (enero de 1997- marzo de 2021) en el Sector de Enfermedades Ampollares del Hospital Ramos Mejía para determinar la epidemiología, la clínica y la terapéutica de esta enfermedad.Diseño: estudio retrospectivo descriptivo y observacional, en el que se analizaron las características clínicas e inmunopatológicas de 34 pacientes con diagnóstico de PM atendidos en el Servicio de Dermatología del Hospital Ramos Mejía desde enero de 1997 hasta marzo de 2021. Materiales y métodos: mediante las historias clínicas y los regis-tros iconográficos, se evaluaron las siguientes variables: prevalencia del diagnóstico de PM en los pacientes atendidos en el Sector, sexo, edad, antecedentes personales, mucosas afectadas, tiempo de evolución hasta el diagnóstico, hallazgos en la IFD, seguimiento clínico y tratamientos instaurados. Resultados: se estudió la evolución clínica de 34 pacientes diagnosticados con PM (5,3% del total de pacientes evaluados en el Sector de Patologías Ampollares). El sexo más afectado fue el femenino y la edad promedio en el momento del diagnóstico fue de 64 años. El 70,6% de los pacientes presentaron comorbilidades asociadas como hipertensión e hipotiroidismo. La mayoría refirió algún evento emocional como factor desencadenante. El sitio más comprometido fue la mucosa ocular y la cavidad oral fue la segunda en frecuencia. El tiempo de evolución promedio hasta el momento del diagnóstico fue de 4 años y 11 meses. El hallazgo más frecuente en la IFD fue la IgG lineal. El 17,6% de los pacientes interrumpieron el seguimiento clínico. El tratamiento más utilizado fue el mofetil micofenolato, con el que se obtuvo buena respuesta terapéutica. Conclusiones: el PM es una enfermedad autoinmune infrecuente que compromete las mucosas y, ocasionalmente, la piel. En este estudio, se observó que la principal mucosa afectada fue la conjuntival, a diferencia de lo referido en la bibliografía internacional dermatológica. El diagnóstico interdisciplinario temprano es fundamental para evitar las secuelas irreversibles.

Introduction: mucous membrane pemphigoid (MMP), also known as benign mucous membrane pemphigoid, cicatricial or mucosynechial pemphigoid, belongs to an heterogeneous group of chronic inflammatory autoimmune blistering diseases, which involves the mucous membranes (oral, ocular, pharyngeal, nasal, esophageal, laryngeal and anogenital) and/ or skin with tendency to scar formation. There are autoantibodies against different components of the basement membrane zone (BPAG 1- BPAG2, Integrin α6ß4, Laminin 332, Col VII, among others). The direct immunofluorescence (DIF) will be of paramount importance, as well as the clinical diagnosis.Objective: review the cases diagnosed with mucous membrane pemphigoid for 24 years (January 1997- March 2021) in the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital to establish the epidemiologic, clinic presentation and available treatments in this pathology.Design: retrospective descriptive and observational study of the clinical and immunopathological characteristics of 34 patients with MMP that were treated at the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital between January 1997 and March 2021.Materials and methods: though the medical histories and the photographic registries, we evaluated the following variables: prevalence of MMP within the patients that came to consult at the Blistering Disease Clinic at the Dermatology Department, sex, age, personal history, the affected mucous, evolution time until the diagnosis, direct immunofluorescence findings, clinical follow-ups and treatments.Results: we studied the clinical evolution of 34 patients diagnosed with MMP at our institution (5.3% from the total of patients at the Blistering Disease Clinic).The most affected gender was female and the average age at diagnosis was 64 years. 70.6% presented comorbidities such as hypertension and hypothyroidism. Most of our patients referred an emotional triggering event. The most affected membrane mucous was the ocular one and the oral was the second one. The delay in diagnosis was 4 years and 11 months. Linear deposits of IgG was the most frequent result in the direct immunofluorescence. 17.6% did not continue clinical follow-up. Mycophenolate mofetil was the most used drug with a good therapeutic response. Conclusions: MMP is a rare autoimmune disease that affects mucous membrane and occasionally the skin. In this study, the ocular involvement was the most frequent one, differing with the international reports. The early interdisciplinary diagnosis is essential to avoid irreversible sequelae.

Penfigoide de membrana mucosa asociado a carcinoma pulmonar ¿penfigoide paraneoplásico? / Mucous membrane penfigoid associated with pulmonary carcinoma ¿paraneoplastic penfigoid?

El penfigoide de la membrana mucosa es una enfermedad autoinmune, que afecta a los diferentes estratos de la mucosa y la piel, y se caracteriza por lesiones vesiculo-ampollosas polimórficas. Presentando combinaciones variables de lesiones orales, oculares, cutáneas, genitales, nasofaríngeas, esofágicas y laríngeas. Su relación con las neoplasias malignas es poco conocida y sigue siendo controvertida. El presente artículo describe el manejo multidisciplinario de una paciente de 46 años de edad que desarrolló un penfigoide de membrana mucosa a nivel de la cavidad oral. Esta patología estuvo asociada a un carcinoma de pulmón, y posteriormente presentó alteraciones multiorgánicas. El tratamiento fue realizado por las especialidades de dermatología, medicina interna, cirugía general, oftalmología, estomatología, cirugía oral y maxilofacial, dicho tratamiento se llevó a cabo por etapas y tuvo una duración de 2 meses aproximadamente, logrando la resolución de las lesiones que presentaba la paciente. Consideramos que se abre una puerta para futuras investigaciones que confirman la relación entre el penfigoide de la membrana mucosa y el carcinoma de pulmón.

Mucous membrane pemphigoid is an autoimmune disease that affects different layers from mucosa and skin, characterized by polymorphic vesicular-bullous lesions, presenting variable combinations of oral, ocular, skin, genital, nasopharyngeal, esophageal, and laryngeal lesions. Its relationship to malignant neoplasms is poorly understood and remains controversial. This article describes the multidisciplinary management of a 46-year-old patient who developed mucous membrane pemphigoid at the oral cavity level. This pathology was associated with lung carcinoma, and later presented multiorgan alterations, for which a treatment was carried out with the specialties of dermatology, internal medicine, general surgery, ophthalmology, stomatology, oral and maxillofacial surgery, said treatment was carried out in stages and lasted for approximately 2 months, achieving the resolution about lesions that patient presented. We believe that a door opens for future research confirming the relationship between mucous membrane pemphigoid and lung carcinoma.

Characteristics Upon Presentation of Ocular Mucous Membrane Pemphigoid Patients in Puerto Rico.

OBJECTIVE: To describe the characteristics upon presentation of a cohort of Hispanic patients living in Puerto Rico with ocular mucous membrane pemphigoid (MMP). METHODS: Retrospective chart review of subjects with ocular MMP at one academic institution and one private practice. Patients with clinical evidence of ocular MMP, along with a positive mucous membrane biopsy revealing linear antibody or C3 deposition in the basement membrane zone, or with a positive indirect immunofluorescence assay were included. Descriptive statistical analysis was performed. RESULTS: Eight patients with ocular mucous membrane pemphigoid were identified. The median age upon presentation was 60.5 years; however, 2 patients were in their 4th decade and one in the 5th decade of life. Females constituted 62.5% of the cohort. All patients presented with stage III ocular MMP in at least one eye and 50% had history of trichiasis. Seven out of eight patients (87.5%) had extraocular symptoms for a median duration of 36 months (range 2-144 months). The most common site of extraocular involvement was the oropharynx, present in 87.5% of patients. CONCLUSION: Our results suggest that in Puerto Rico ocular MMP most commonly presents in the seventh decade of life. The presence of symblepharon, trichiasis or oropharyngeal mucosal disease should prompt further evaluation and consideration for immunopathological tissue analysis and an IIF assay.