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.

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.

Síndrome Sjögren y penfigoide de las membranas mucosas en paciente con antecedente de timoma: Reporte de caso y revisión de literatura / Sjögren Syndrome and Mucous Membrane Pemphigoid in a Patient with a History of Thymoma: Case Report and Literature Review

RESUMEN Los timomas son las neoplasias mediastínicas más comunes en adultos. Los pacientes con timoma son frecuentemente diagnosticados con síndromes paraneoplásicos y generalmente están asociados con varias condiciones autoinmunes. El síndrome de Sjögren (SS) es un trastorno inflamatorio autoinmune, que se caracteriza por la destrucción mediada por linfocitos de las glándulas exocrinas, que conduce a la ausencia de secreción glandular, en particular las salivales y las lagrimales; la hiposalivación puede variar desde una ligera reducción en el flujo de saliva con molestias transitorias, a un grave deterioro de la salud oral y malestar psicológico. El penfigoide de membranas mucosas (PMM) es una enfermedad autoinmune crónica, muestra predominante afectación de la mucosa, afectando con mayor frecuencia la cavidad oral, seguida de la conjuntiva, la cavidad nasal y el esófago; intraoralmente incluye gingivitis descamativa, vesículas, erosiones cubiertas por pseudomembranas y úlceras. El objetivo de este trabajo es presentar el primer caso de una paciente con antecedente de timoma, la cual, conjuntamente presentó SS y PMM; además evidenciar la importancia del manejo multidiciplinario para un diagnóstico preciso y temprano, ya que así, aumenta la eficiencia y la eficacia de la estrategia de tratamiento.

ABSTRACT Thymomas are the most common mediastinal neoplasms in adults. Patients with thymoma are frequently diagnosed with paraneoplastic syndromes and are generally associated with several autoimmune conditions. Sjögren's syndrome (SS) is an autoimmune inflammatory disorder, characterized by the lymphocyte-mediated destruction of the exocrine glands, which leads to the absence of glandular secretion, particularly salivary and lacrimal; hyposalivation can vary from a slight reduction in the flow of saliva with transient discomfort, to a serious deterioration of oral health and psychological distress. Mucous membrane pemphigoid (MMP) is a chronic, autoimmune disease that shows predominant mucosal involvement, most commonly affecting the oral cavity, followed by the conjunctiva, the nasal cavity and the esophagus; intraorally includes scaly gingivitis, vesicles, erosions covered by pseudomembranes and ulcers. The objective of this work is to present the first case of a patient with a history of thymoma, which, jointly presented SS and MMP; also highlight the importance of multidisciplinary management for an accurate and early diagnosis, as this increases the efficiency and efficacy of the treatment strategy.

Mucous membrane pemphigoid presenting as bleeding gums and burning sensation of mouth: a case report

@#Dental practitioners may encounter various oral mucosal diseases. Oral lesions can be the early manifestation of many systemic diseases. Mucous membrane pemphigoid (MMP) is a chronic, auto immune vesiculobullous lesion involving the mucocutaneous structures. It is important for a dental surgeon to be aware of oral presentations of MMP. Early and accurate diagnosis of this rare clinical entity is essential for the effective management of the lesions which may reduce or lessen disease progression. Present case report highlights the clinical presentations of MMP affecting the oral mucosa along with diagnostic features.

An analysis of periodontal status in desquamative gingivitis and the correlative factors / 口腔疾病防治

Objective @#To study the clinical periodontal status of patients with desquamative gingivitis (DG) and analyze the factors that influence clinical periodontal indicators.@*Methods @#A purposive sampling method was used to obtain 42 subjects for a DG case group and a control group. Periodontal clinical indicators were detected, and related factors were analyzed. @*Results @#The DG patients were primarily middle-aged women. Periodontal clinical indicators were more prevalent in individuals with oral lichen planus (OLP) and mucous membrane pemphigoid (MMP) than in the control group. Probing depth (PD) (χ2=53.058, P<0.001; χ2=32.989, P<0.001), clinical attachment (χ2=30.292, P<0.001; χ2=32.470, P<0.001) and the positive rate of bleeding on probing (BOP) (χ2=50.003, P<0.001; χ2=36.236, P<0.001) were higher in the OLP and MMP group than in the control group. The time interval between the onset and treatment of DG was correlated with PD (rs=0.523, P<0.001) and the rate of positive BOP sites (rs=0.377, P=0.014). @*Conclusion@# Patients with DG have obvious periodontal lesions. Early medical intervention is helpful for diagnosing and treating DG-related oral and systemic disease.

Desenlaces en pacientes operados con queratoprótesis boston tipo 1 en un centro oftalmológico especializado en la ciudad de Medellín / Outcomes in patients with boston type 1 keratoprosthesis in a specialized center in Medellín

Objetivo: reportar las características y descenlaces de pacientes con queratoprótesis Boston tipo 1. Diseño: estudio observacional descriptivo de corte transversal. Método: se incluyeron los pacientes operados con queratoprótesis Boston tipo 1 residentes en Colombia, mayores de 17 años, sin queratoprótesis previas y que tuviera más de 6 meses de seguimiento, para un total de 39 ojos de 39 pacientes. La cirugía se indicó en los casos con o sin queratoplastia previa que tuvieran agudeza visual (AV) igual o inferior a cuenta dedos y déficit de limbo bilateral o vascularización corneal profunda en más de 3 cuadrantes. Se recolectó y analizó la información de la historia clínica en forma retrospectiva y prospectiva. Las variables principales fueron: agudeza visual, retención del dispositivo y complicaciones. Se realizó un análisis univariado presentando los resultados en frecuencias absolutas y porcentajes para las variables categóricas y para las variables cuantitativas como promedio y desviación estándar (DE) si tienen distribución normal o como mediana y rango intercuartil si no tienen distribución normal. Resultados: La mediana de seguimiento fue de 53.5 meses (rango 6 meses a 10 años). La mayoría de pacientes (66.7%) habían tenido previamente queratoplastia penetrante. El diagnóstico de base fue en 53.8% condiciones no inflamatorias yen 46.2% condiciones inflamatorias. La AV preoperatoria en todos los ojos estaba entre percepción de luz y cuenta dedos; posterior a la cirugía el 97.4% tuvieron mejoría de la AV. Para el fi nal del seguimiento la AV permaneció mejor que la preoperatoria en 53.8%, fue igual a esta en 17.94% y peor que esta en 28.2%. Hubo necrosis periprostética en 28.2%, y 38.5% tuvieron recambio del dispositivo. Al final del seguimiento 89.7% de los pacientes tenían una queratoprótesis in-situ. Conclusión: la queratoprótesis Boston tipo 1 es una opción viable para mejorar la AV en pacientes con trasplantes de córnea fallidos o en quienes se prevé alta tasa de fallo o rechazo de este. En la gran mayoría de los pacientes se logra mejoría inicial significativa de la AV, la cual se mantiene al final del seguimiento en más de la mitad de ellos. Refinamientos progresivos en la técnica quirúrgica y el manejo postoperatorio disminuirán las complicaciones que llevan a la perdida visual progresiva

Purpose: to report characteristics and outcomes in patients with Boston type 1 keratoprosthesis. Design: observational descriptive cross-sectional study. Method: Patients with Boston type 1 queratoprosthesis who were living in Colombia, 17 years or older, without previous keratoprosthesis and with more than 6 months follow up were included, for a fi nal of 39 eyes from 39 patients. Surgery was indicated for those with or without previous keratoplasty who had visual acuity (VA) of counting fi ngers or worse and bilateral limbal stem cell deficiency or deep corneal vascularization in more than 3 quadrants. Information was collected retrospectively and prospectively from medical registries and was analyzed. Main variables were: visual acuity, device retention and complications. An univariate analysis was conducted and results are presented in absolute frequencies and percentages for categoric variables and for quantitative variables in average and standard deviation (SD) if they have normal distribution and in median and interquartile range if they do not have normal distribution. Results: Median follow up was 53.5 months (range 6 months to 10 years). Most patients (66.7%) had previous penetrating keratoplasty. Baseline diagnoses were in 53.8% non-infl ammatory conditions and in 46.2% inflammatory conditions. Preoperative VA ranged between light perception and counting fingers in all eyes; aft er surgery 97.4% had improvement in VA. By the end of follow up VA remained better than preoperative in 53.8%, was the same as preoperative in 17.94% and worse than preoperative in 28.2%. There was periprosthetic necrosis in 28.2%, and 38.5% had keratoprothesis exchange. At the end of follow up 89.7% of patients had a keratoprosthesis in-situ. Conclusion: Boston type 1 keratoprosthesis is a viable option to improve VA in patients with previously failed corneal transplants or in cases that anticipate bad prognosis for keratoplasty. In most patients there was a signifi cant initial improvement in vision, and more than half of them retained better than initial VA on their last follow up. Further refinements in surgical technique and postoperative care would decrease complications that lead to progressive visual loss.

Systemic corticosteroid in the treatment of a 9-year-old boy with oral mucous membrane pemphigoid: a case report / Utilização de corticóide sistêmico no tratamento do penfigóide benigno das membranas mucosas em uma criança de 9 anos de idade: um relato de caso

Objective: The aim of this report is present a rare case of oral Mucous Membrane Pemphigoid (MMP) in a 9-year-old-boy successfully treated with low doses of systemic corticosteroid. Case description: A 9-year-old boy was referred to our service with the complaint of painful gingiva and generalized burning mouth sensation over 1 year. Intraoral examination revealed desquamative lesions affecting the entire attached gingiva of both the maxilla and the mandible. After the incisional biopsy the MMP diagnosis was confirmed, and the patient was initially treated with dexamethasone 0,1mg/Ml mouth rinse twice daily for 24 weeks. Due the difficulty in eating and the presence of persistent large oral lesions, systemic prednisone 20 mg (1 mg/Kg) was prescribed for 4 weeks with a 5 mg gradual reduction per week over 3 weeks. After initiating the systemic corticosteroid therapy, the patient showed total regression of the lesions, and no indication of recurrence has been observed in the past 6 months. Conclusion: There is no cure for MMP; however, treatment can provide complete and long-lasting remission. Based on this premise, it is plausible to consider not only topical but also low doses of systemic corticosteroids in resistant cases of oral MMP, even in children. These measures could improve the quality of life of these patients by reducing pain and, consequently, improving the child's eating behavior.

Objetivo: O objetivo deste relato é apresentar um caso raro de Penfigóide Benigno das Membranas Mucosas (PBMM) em um menino de 9 anos de idade tratado com sucesso com baixas doses de corticosteróide sistémico. Relato de caso: Um menino de 9 anos de idade foi encaminhado ao nosso serviço com a queixa de gengiva dolorida e sensação de queimação na boca há aproximadamente 1 ano. Ao exame físico intraoral constatou-se a presença de lesões descamativas que afetavam toda a gengiva inserida, tanto na maxila quanto na mandíbula. Após a realização de uma biópsia incisional o diagnóstico de PBMM foi confirmado, e o paciente foi inicialmente tratado com elixir de dexametasona 0,1mg / ml duas vezes por dia durante 24 semanas. Devido a dificuldade em comer e a presença de grandes lesões orais persistentes, foi prescrito prednisona sistêmica 20 mg (1 mg / Kg) por 4 semanas com uma redução gradual de 5 mg por semana durante 3 semanas. Depois de iniciar a corticoterapia sistêmica, o paciente apresentou regressão total das lesões, e nenhum sinal de recorrência tem sido observado nos últimos 6 meses. Conclusão: Não há cura para o PBMM; no entanto, o tratamento pode proporcionar a remissão completa e de longa duração. Com base nesta premissa, é plausível considerar a terapia tópica, mas também a utilização de corticosteroides sistêmicos em casos resistentes de PBMM oral, mesmo em crianças. Estas medidas poderiam melhorar a qualidade de vida desses pacientes, reduzindo a dor e, consequentemente, melhorando a alimentação da criança.

Manifestações periodontais associadas às doenças auto-imunes: uma revisão de literatura / Periodontal manifestations associated with autoimmune diseases: a literature review

As doenças auto-imunes envolvem a formação de auto-anticorpos direcionados contra alguns elementos teciduais, particularmente os da pele ou superfícies das mucosas, como forma sistêmica. Dentre estas doenças podemos destacar o pênfigo vulgar, o lúpus eritematoso sistêmico e o penfigóide das membranas mucosas. Estas estão, muitas vezes, associadas com sintomas bucais, podendo apresentar em diversos sítios orais a presença de lesões vesículo-bolhosas e gengivite descamativa. Baseado nisto, este artigo realizou uma revisão de literatura que teve como objetivo associar as manifestações periodontais ao pênfigo vulgar, lúpus eritematoso sistêmico e penfigóide das membranas mucosas com o intuito fornecer aos profissionais um melhor entendimento sobre essas doenças, de modo que eles sejam capazes de oferecer um atendimento clínico mais adequado para estes pacientes. Os resultados puderam mostrar que há uma associação dessas doenças auto-imunes a manifestações periodontais, especialmente a gengivite descamativa. Além disso, a literatura as associa à perda óssea alveolar, ao aumento do sangramento gengival, a perda de inserção e a outras desordens periodontais.

Penfigoide de membranas mucosas ocular: a propósito de un caso clínico / Ocular mucous membrane pemphigoid: a case report

El Penfigoide de membranas mucosas (PMM), corresponde a un tipo de enfermedad ampollosa autoinmune, la que se caracteriza por presentar una alteración en la adhesión de las estructuras cutáneas. Su presentación ocular corresponde a una conjuntivitis crónica de difícil manejo que puede comprometer severamente la superficie ocular con formación de simbléfaron, acortamiento del fórnix, conjuntivalización y neovascularización corneal, conduciendo a la amaurosis total si no es tratado ni diagnosticado oportunamente. El diagnóstico por medio de biopsia de mucosa oral y conjuntival con estudio histológico y de inmunofluorescencia directa (IFD), aumenta considerablemente la sensibilidad y permite iniciar un tratamiento inmunosupresor sistémico disminuyendo la tasa de progresión de la enfermedad. (AU)

Mucous membrane pemphigoid (MMP), a type of autoimmune blistering disease, is characterized by an alteration in the adherence of skin structures. Ocular manifestations include chronic conjunctivitis that can severely compromise the ocular surface with symblepharon formation , shortening of the fórnix , conjunctivalization and corneal neovascularization , leading to total blindness if it is not diagnosed and treated promptly. The biopsy of oral and conjunctival mucosa for histological study and direct immunofluorescence (DIF) significantly increases sensitivity and allows the use of systemic immunosuppressive therapy to avoid the progression of the disease.(AU)

Mucous membrane pemphigoid with antibodies against β3 subunit of laminin-332: First report from India.

Mucous membrane pemphigoid (MMP) is a chronic, recurrent, progressive, subepidermal blistering disorder, mainly affecting the mucous membranes. Anti-laminin 332 MMP is a distinct subset of MMP with antibodies, mainly targeted against α3 or γ2 subunit. Antibodies exclusively against β3 subunit are rarely seen. An internal malignancy is frequently associated with anti-laminin 332 MMP. This disorder usually responds poorly to treatment, requiring multidisciplinary approach. Herein, we describe the first case of anti-laminin-332 MMP from India, which showed antibodies exclusively to β3 subunit.