Authors Reply to Letter to the Editor - In Response to: Comment on Bocanegra-Oyola et al.'s "Clinical Characteristics of Ocular Mucous Membrane Pemphigoid: A Systematic Review and Meta-Analysis".

In response to Dr. Kasperkiewicz's commentary on our meta-analysis conducted by Bocanegra-Oyola et al., we fully agree with refining diagnostic processes for ocular pemphigoid, particularly in differentiating it from pseudopemphigoid. We concur that relying solely on clinical findings may result in misdiagnoses. Confirming the diagnosis via biopsy can be challenging, requiring multiple biopsies in some patients, and should always be supported by a multidisciplinary clinical assessment involving ophthalmologists and dermatologists.

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.

Ocular cicatricial pemphigoid: is there an association with autoimmune diseases?

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.

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.

Ocular Cicatricial Pemphigoid, Sjögren's Syndrome, and Hashimoto's Thyroiditis as a Multiple Autoimmune Syndrome: A case report.

PURPOSE: To present a rare and novel association of Ocular Cicatricial Pemphigoid, Sjögren's Syndrome, and Hashimoto's Thyroiditis as a Multiple Autoimmune Syndrome. CASE REPORT: A 43-year-old Colombian female, presented with corneal ulcers, associated with trichiasis. At the ophthalmological examination forniceal shortening OU and symblepharon OD was found. Conjunctival biopsy was performed, evidencing linear deposition of IgG and IgA antibodies along the basement membrane of the conjunctiva, confirming Ocular Cicatricial Pemphigoid diagnosis. After 12 years, the patient presented constitutional symptoms, xerostomia, and worsening of xerophthalmia. Laboratory tests showed positive Anti-TG, Anti-TPO, Anti-Ro, and Anti-La antibodies, and salivary gland biopsy was consistent with Sjögren's Syndrome. Due to these findings, Hashimoto's Thyroiditis and Sjögren's Syndrome were diagnosed, defining a Multiple Autoimmune Syndrome. CONCLUSION: A novel association of Multiple Autoimmune Syndrome is presented in this case. Ophthalmologists and other specialists involved in the evaluation and treatment of patients with autoimmune diseases, should be aware of this clinical presentation. A multidisciplinary approach in this condition is important for optimum treatment instauration and follow-up, in order to prevent complications.

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.

Incidence, prevalence, and demographic characteristics of ocular cicatricial pemphigoid in Colombia: data from the National Health Registry 2009-2019.

AIM: To provide an epidemiological characterization of ocular cicatricial pemphigoid (OCP) in Colombia. METHODS: We conducted a cross-sectional study using SISPRO. We applied the specific code of the International Classification of Diseases for Ocular Pemphigoid, from 2009-2019 to estimate prevalence, incidence, and the demographic status of the disease in Colombia. RESULTS: The estimated average prevalence was 0.22 per 1 000 000 inhabitants, and the estimated average incidence was 0.24 per 1 000 000 inhabitants. With a female predominance of 62.5%, and a male/female ratio of 1:1.6. The group of patients diagnosed with the disease after the age of 80 presented the highest prevalence. The departments with the highest prevalence were Antioquia, Bogotá, and Santander. CONCLUSION: There are important differences between worldwide and Colombian prevalence and incidence data, which may be related to genetic and epigenetic factors, and the possible underdiagnosis of the disease. According to the results, OCP is an extremely rare disease in Colombia. Nevertheless, it is important to encourage awareness of the disease due to its devastating consequences.

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.

Ectodermal Dysplasia: Association with Anti-Basement Membrane Autoantibodies.

Ectodermal dysplasia (ED) is a group of several genetic conditions with absence or dysgenesis of at least two ectodermal derivatives: teeth, skin and its appendages including hair, nails, eccrine and sebaceous glands. The most important clinical findings in patients with ED are hypodontia, hypotrichosis, and hypohidrosis, which can lead to episodes of hyperthermia. Few reports have focused on the progressive keratopathy in ED. Cicatrizing conjunctivitis associated with anti-basement membrane autoantibodies has been described. We report a series of three ectodermal dysplasia patients with an ocular phenotype typically seen in ocular mucous membrane pemphigoid; conjunctival immunohistopathology revealed anti-basement membrane autoantibodies in all of them, and systemic immunosuppression proved to be effective in improving symptoms and helping to stabilize ocular surface disease.

Aspectos epidemiológicos y opciones de tratamiento para las complicaciones oftalmológicas de las enfermedades mucosinequiantes / Epidemiology and treatment options for ophthalmic complications of mucosinequiant diseases

Introducción: Las enfermedades mucosinequiantes son aquellas que afectan piel y mucosas con lesiones vesículo-ampollar, generalmente son bilaterales y se caracterizan por distorsionar la anatomía de la conjuntiva, párpados, vías lagrimales y universalmente conducen a queratoconjuntivitis seca. Objetivo: Actualizar aspectos epidemiológicos y opciones de tratamiento para las complicaciones oftalmológicas de las enfermedades mucosinequiantes. Material y Métodos: Se realizó una revisión bibliográfica sobre aspectos conceptuales, clasificación, etiopatogenia, epidemiología, cuadro clínico, complicaciones y tratamiento oftalmológico de las enfermedades mucosinequiantes en la literatura impresa y en soporte digital disponible. Utilizamos para la búsqueda electrónica bases de datos como PubMed, BioMed Central y SCIELO, abarcando varios años hasta el presente. Abordamos enfermedades como Tracoma, Penfigoide cicatrizal, Síndrome de Stevens-Johnson y Conjuntivitis leñosa, por ser de las enfermedades mucosinequiantes las que mayor compromiso conjuntival presentan.Resultados: Las enfermedades mucosinequiantes producen cicatrización conjuntival y graves complicaciones oftalmológicas que pueden comprometer la visión hasta llegar a la ceguera. Conclusiones: Las enfermedades mucosinequiantes son causa frecuente de morbilidad ocular por el severo daño que ocasionan a la superficie ocular. El uso de injerto de membrana amniótica, mucosa bucal y trasplante de células madre del limbo córneo conjuntival son opciones utilizadas con buenos resultados en el tratamiento de las complicaciones oftalmológicas de estas enfermedades(AU)

Introduction: mucosinequiant diseases are those that affect skin and mucosa with blister-vesicular wounds, they are usually bilateral and are characterized by distorting the conjunctival anatomy, eyelids, lachrymal way and that universally lead to a dry keratoconjunctivitis. Objective: to update epidemiological issues and treatment options for ophthalmic complications of mucosinequiant diseases. Material and Methods: a literature review on conceptual issues, classification, etiopathology, epidemiology, clinical manifestation, complications and treatment of mucosinequiant ophthalmological diseases available in hard copies and digital support was made. To search, we used electronic databases such as PubMed, Central BioMed and SCIELO, extending over several years to the present. We deal with diseases such as trachoma, cicatricial pemphigoid, Stevens-Johnson syndrome and woody conjunctivitis, because mucosinequiant diseases are those which present greater conjunctival compromise. Results: mucosinequiant cause conjunctival cicatrization and serious ophthalmologic complications that could compromise vision up to blindness. Conclusions: mucosinequiant diseases are a frequent cause of ocular morbidity causing severe damage to the ocular surface. The use of amniotic membrane graft, oral mucosa and corneal conjunctival limbus stem cell transplant are used options with good results in the treatment of ophthalmic complications of these diseases(AU)

Tratamiento sistémico del penfigoide cicatrizal ocular / Systemic treatment of ocular cicatricial pemphigoid

El penfigoide cicatrizal ocular (PCO) es una enfermedad ampollar autoinmune que produce daño conjuntival grave. Se conoce poco acerca de la respuesta del PCO al tratamiento inmunosupresor. Describimos un grupo de 76 pacientes con PCO, 62 mujeres y 14 hombres. La edad media al diagnóstico fue de 67 ñ 14 años, con un retraso de 7.5 ñ 10 años. Sesenta se siguieron en nuestro servicio por 19 ñ 21 meses. De 51 en quienes se describe la gravedad de la enfermedad al inicio del tratamiento, fue leve en 19 pacientes, moderada en 19, grave en cinco y muy grave en ocho. Las drogas mayormente prescriptas fueron dapsona en 35 pacientes, de los que 23 la discontinuaron por efectos adversos, y metotrexate en 42, de los que nueve lo suspendieron. Otros recibieron azatioprina, ciclofosfamida y ciclosporina. A 17 se les indicaron corticoides orales, además del inmunosupresor. Cuatro combinaron dos drogas para controlar la enfermedad. Tres pacientes refractarios recibieron gammaglobulina EV con buena respuesta. De 48 evaluados, 39 mostraron mejoría, ocho no tuvieron cambios y uno progresó. En nuestra experiencia, metotrexate y azatioprina son efectivos, con baja toxicidad. Dapsona es útil en casos leves, con efectos adversos frecuentes. La gammaglobulina EV fue efectiva en casos refractarios.(AU)

Ocular cicatricial pemphigoid (OCP) is a blistering autoimmune disease that can produce severe conjunctival damage. Its response to immunosuppressive treatment is poorly known. We describe a group of 76 patients, 62 women and 14 men. Mean age at diagnosis was 67ñ14 years old, with a delay to diagnosis of 7.5ñ10 years. Sixty patients continued their follow up in our services for 19ñ21 months. Nineteen out of 51 had mild disease, 19 moderate, 5 severe and 8 very severe at onset of treatment. The more frequently prescribed drugs were dapsone, in 35 (23 discontinued it because of adverse effects), and methotrexate in 42 patients, nine of them stopped it. Other patients received azathioprine, cyclophosphamide and ciclosporine. Seventeen received oral steroids in addition to immunosuppresive drugs. Four patients combined two immunosupressive drugs to control their disease. In three refractory cases IV immunoglobulin (Ig) was administered with good response. From 48 evaluated patients, 39 improved with treatment, eight remained stable and one progressed. In our experience, methotrexate and azathioprine were effective drugs, with low toxicity. Dapsone was useful in mild cases, with frequent adverse effects. IVIg was effective for refractory cases.(AU)

Tratamiento sistémico del penfigoide cicatrizal ocular / Systemic treatment of ocular cicatricial pemphigoid

El penfigoide cicatrizal ocular (PCO) es una enfermedad ampollar autoinmune que produce daño conjuntival grave. Se conoce poco acerca de la respuesta del PCO al tratamiento inmunosupresor. Describimos un grupo de 76 pacientes con PCO, 62 mujeres y 14 hombres. La edad media al diagnóstico fue de 67 ± 14 años, con un retraso de 7.5 ± 10 años. Sesenta se siguieron en nuestro servicio por 19 ± 21 meses. De 51 en quienes se describe la gravedad de la enfermedad al inicio del tratamiento, fue leve en 19 pacientes, moderada en 19, grave en cinco y muy grave en ocho. Las drogas mayormente prescriptas fueron dapsona en 35 pacientes, de los que 23 la discontinuaron por efectos adversos, y metotrexate en 42, de los que nueve lo suspendieron. Otros recibieron azatioprina, ciclofosfamida y ciclosporina. A 17 se les indicaron corticoides orales, además del inmunosupresor. Cuatro combinaron dos drogas para controlar la enfermedad. Tres pacientes refractarios recibieron gammaglobulina EV con buena respuesta. De 48 evaluados, 39 mostraron mejoría, ocho no tuvieron cambios y uno progresó. En nuestra experiencia, metotrexate y azatioprina son efectivos, con baja toxicidad. Dapsona es útil en casos leves, con efectos adversos frecuentes. La gammaglobulina EV fue efectiva en casos refractarios.

Ocular cicatricial pemphigoid (OCP) is a blistering autoimmune disease that can produce severe conjunctival damage. Its response to immunosuppressive treatment is poorly known. We describe a group of 76 patients, 62 women and 14 men. Mean age at diagnosis was 67±14 years old, with a delay to diagnosis of 7.5±10 years. Sixty patients continued their follow up in our services for 19±21 months. Nineteen out of 51 had mild disease, 19 moderate, 5 severe and 8 very severe at onset of treatment. The more frequently prescribed drugs were dapsone, in 35 (23 discontinued it because of adverse effects), and methotrexate in 42 patients, nine of them stopped it. Other patients received azathioprine, cyclophosphamide and ciclosporine. Seventeen received oral steroids in addition to immunosuppresive drugs. Four patients combined two immunosupressive drugs to control their disease. In three refractory cases IV immunoglobulin (Ig) was administered with good response. From 48 evaluated patients, 39 improved with treatment, eight remained stable and one progressed. In our experience, methotrexate and azathioprine were effective drugs, with low toxicity. Dapsone was useful in mild cases, with frequent adverse effects. IVIg was effective for refractory cases.

Tratamiento sistémico del penfigoide cicatrizal ocular / Systemic treatment of ocular cicatricial pemphigoid

El penfigoide cicatrizal ocular (PCO) es una enfermedad ampollar autoinmune que produce daño conjuntival grave. Se conoce poco acerca de la respuesta del PCO al tratamiento inmunosupresor. Describimos un grupo de 76 pacientes con PCO, 62 mujeres y 14 hombres. La edad media al diagnóstico fue de 67 ± 14 años, con un retraso de 7.5 ± 10 años. Sesenta se siguieron en nuestro servicio por 19 ± 21 meses. De 51 en quienes se describe la gravedad de la enfermedad al inicio del tratamiento, fue leve en 19 pacientes, moderada en 19, grave en cinco y muy grave en ocho. Las drogas mayormente prescriptas fueron dapsona en 35 pacientes, de los que 23 la discontinuaron por efectos adversos, y metotrexate en 42, de los que nueve lo suspendieron. Otros recibieron azatioprina, ciclofosfamida y ciclosporina. A 17 se les indicaron corticoides orales, además del inmunosupresor. Cuatro combinaron dos drogas para controlar la enfermedad. Tres pacientes refractarios recibieron gammaglobulina EV con buena respuesta. De 48 evaluados, 39 mostraron mejoría, ocho no tuvieron cambios y uno progresó. En nuestra experiencia, metotrexate y azatioprina son efectivos, con baja toxicidad. Dapsona es útil en casos leves, con efectos adversos frecuentes. La gammaglobulina EV fue efectiva en casos refractarios.(AU)

Ocular cicatricial pemphigoid (OCP) is a blistering autoimmune disease that can produce severe conjunctival damage. Its response to immunosuppressive treatment is poorly known. We describe a group of 76 patients, 62 women and 14 men. Mean age at diagnosis was 67±14 years old, with a delay to diagnosis of 7.5±10 years. Sixty patients continued their follow up in our services for 19±21 months. Nineteen out of 51 had mild disease, 19 moderate, 5 severe and 8 very severe at onset of treatment. The more frequently prescribed drugs were dapsone, in 35 (23 discontinued it because of adverse effects), and methotrexate in 42 patients, nine of them stopped it. Other patients received azathioprine, cyclophosphamide and ciclosporine. Seventeen received oral steroids in addition to immunosuppresive drugs. Four patients combined two immunosupressive drugs to control their disease. In three refractory cases IV immunoglobulin (Ig) was administered with good response. From 48 evaluated patients, 39 improved with treatment, eight remained stable and one progressed. In our experience, methotrexate and azathioprine were effective drugs, with low toxicity. Dapsone was useful in mild cases, with frequent adverse effects. IVIg was effective for refractory cases.(AU)

A importância do diagnóstico precoce do penfigoide cicatricial: relato de caso / The importance of early diagnosis in cicatricial pemphigoid: case report

O penfigóide cicatricial é uma doença bolhosa inflamatória de caráter crônico e autoimune que afeta primariamente as superfícies mucosas. Auto anticorpos são produzidos contra o complexo de adesões dos hemidesmossomas da membrana basal da pele e mucosas. Afeta a cavidade bucal, laringe esôfago, membrana ocular e raramente a pele. A formação de cicatrizes é característica e pode resultar em cegueira, quando envolve a conjuntiva ocular. O envolvimento da mucosa bucal é observado na maioria dos pacientes e, quando acomete a gengiva, produz um quadro de gengivite descamativa. A etiologia é desconhecida, no entanto tem sido relatada a indução por drogas como possível desencadeador da doença. O diagnóstico é obtido com base na história clínica, biópsia e imunofluorescência direta. O tratamento é feito através de corticosteroide tópico ou sistêmico, na dependência da gravidade da doença. O presente trabalho tem por objetivo relatar um caso de penfigoide cicatricial onde a paciente apresentava apenas lesões bucais, tendo sido estabelecido o diagnóstico por meio da histopatologia através da microscopia óptica a qual apresentou aspecto bastante característico. O Tratamento intituído foi o uso de corticosteroide sistêmico. A paciente está em tratamento, com evolução satisfatória e compatível com o diagnóstico.

The cicatricial pemphigoid is a bullous disease of character chronic inflammatory and autoimmune diseases primarily affecting the mucosal surfaces. Auto-antibodies are produced against the hemidesmosomal complex of members of the basal membrane of the skin and mucous membranes. Affects the oral cavity, larynx, esophagus, eye membrane and rarely the skin. The formation of scar tissue is characteristic and can result in blindness if it involves the ocular conjunctiva. The involvement of the oral mucosa is observed in most patients and when affects the gingiva produces a table of gingivitis peel. The etiology is unknown, however, has been reported to be induced by drugs as a possible trigger of the disease. The diagnosis is made based on clinical history, biopsy and direct immunofluorescence. Treatment is realized by topical or systemic corticosteroids, depending on the severity of the disease, This study aims to report a case of cicatricial pemphigoid in which the patient had oral lesions only was the diagnosis established by histopathology by light microscopy which showed very characteristic appearance. The treatment was the use of systemic corticosteroids. The patient is in treatment, with satisfactory evolution and is compatible with the disgnosis.