RESUMO
Epitope spreading often occurs in patients with autoimmune bullous diseases (ABDs), resulting in exposure of more antigenic epitopes, aggravation or transformation of pre-existing diseases, or concurrence of other diseases. With the increase in the immunological evidence for epitope spreading, more and more scholars have realized that epitope spreading plays an important role in the development of ABDs. This review introduces the phenomenon of epitope spreading in ABDs from 4 aspects, including the concurrence of or transformation between different types of pemphigoid, different types of pemphigus, pemphigus and pemphigoid, as well as between ABDs and other skin diseases.
RESUMO
RESUMEN Las enfermedades ampollares autoinmunes (EAA) comprenden un conjunto heterogéneo de patologías de baja frecuencia de presentación con evolución crónica, remisiones, exacerbaciones y una importante morbimortalidad aun con tratamiento temprano y adecuado. Existen pocos estudios epidemiológicos en nuestra población que incluyan todo el espectro de enfermedades ampollares. Objetivos : Determinar las características clínico-epidemiológicas de las EAA, describir tratamientos realizados, tiempo de control de actividad de la enfermedad y tiempo desde iniciado el tratamiento hasta la remisión completa. Determinar recaídas de las patologías estudiadas, evaluando si existe relación entre las mismas y algún factor reconocido. Materiales y métodos : Estudio retrospectivo y descriptivo. Se incluyeron todos los pacientes con diagnóstico clínico, histopatológico y de inmunofluorescencia de las EAA que hayan tenido seguimiento por al menos un año en el servicio de dermatología del HPUC, en el período de enero de 2008 hasta noviembre de 2018. Resultados : Se incluyeron 53 pacientes de los cuales el 50,9% tenía Penfigoide Ampollar (PA), 22,6% Dermatitis Herpetiforme (DH) y 17% de Pénfigo vulgar (PV). La edad de los pacientes se encontró entre 21 y 90 años. Las ampollas tensas y erosiones fueron las presentaciones más comunes. Los tratamientos sistémicos se realizaron en el 74% de los casos. Registramos recaídas en el 63% de los casos y dos casos de muerte durante el tratamiento. Se estableció más frecuencia de enfermedad cardiovascular en los pacientes con PA. Conclusiones : Es el primer trabajo retrospectivo sobre EAA en nuestro medio. Nuestros hallazgos permiten determinar una frecuencia importante de PA en la consulta dermatológica y de DH, hecho no reportado en otros estudios con poblaciones similares.
ABSTRACT The autoimmune bullous diseases (ASD) comprise a heterogeneous group of pathologies with a low frequency of presentation that presents a chronic evolution, with remissions, exacerbations and an important morbidity and mortality even with early and adequate treatment. There are few epidemiological studies in our population that include the entire spectrum of bullous diseases. Objectives : To determine the clinical-epidemiological characteristics of AAS, describe treatments performed, control time of disease activity and time from treatment initiation to complete remission. Determine relapses of the studied pathologies, evaluating if there is a relationship between them and some recognized factor. Materials and methods : Retrospective and descriptive study. All patients with clinical, histopathological and immunofluorescence diagnosis of the AAS who had followed up for at least one year in the dermatology service of the HPUC, from January 2008 to November 2018 were included. Results: 53 were included. patients of which 50.9% had Bullous Pemphigoid (BP), 22.6% Dermatitis herpetiformis (DH) and 17% Pemphigus Vulgaris (PV). The age of the patients was between 21 and 90 years. Tense blistering forms and erosions were the most common forms of presentation. Systemic treatments were performed in 74% of the cases. We recorded relapses in 63% of the cases and two cases of death during the treatment. More frequency of cardiovascular disease was established in patients with BP. Conclusions : It is the first retrospective work on EAA in our environment. Our findings allow determining an important frequency of PA in the dermatological consultation and DH, fact this last, not reported in other studies with similar populations.
RESUMO
Background: The subepidermal autoimmune bullous diseases are a subset of immunobullous diseases encountered less frequently in the Indian population. There is a paucity of data on the prevalence, demographic and clinicopathological spectrum of various subepidermal autoimmune bullous diseases from India. Aim: To determine the demographic and clinicopathological profi le of subepidermal autoimmune bullous diseases in Indian patients, presenting to the Immunobullous Disease Clinic of Postgraduate Institute of Medical Education and Research, Chandigarh. Methods: Patients seen from November 2013 to November 2014 who fulfi lled the preset diagnostic criteria of subepidermal autoimmune bullous diseases were identifi ed from case records. Data regarding demographic characteristics, clinical profi le, immunopathological fi ndings and treatment were collected from the predesigned proforma. Results: Of 268 cases of autoimmune bullous diseases registered, 50 (18.7%) were subepidermal autoimmune bullous diseases. Bullous pemphigoid was most frequently seen in 20 (40%) cases, followed by dermatitis herpetiformis in 14 (28%), mucous membrane pemphigoid in 6 (12%), chronic bullous dermatosis of childhood / linear immunoglobulin A bullous dermatosis in 5 (10%), lichen planus pemphigoides in 3 (6%), pemphigoid gestationis and epidermolysis bullosa acquisita in 1 (2%) case each. None of the patients had bullous systemic lupus erythematosus. Limitations: We could not perform direct and indirect immunofl uorescence using salt-split skin as a substrate and immunoblotting due to non-availability of these facilities. Therefore, misclassifi cation of subepidermal autoimmune bullous diseases in some cases cannot be confi dently excluded. Conclusion: Subepidermal autoimmune bullous diseases are not uncommon in Indian patients. Bullous pemphigoid contributes maximally to the burden of subepidermal autoimmune bullous diseases in India, similar to that in the West, although the proportion is lower and disease onset is earlier. Dermatitis herpetiformis was observed to have a higher prevalence in our population, compared to that in the West and the Far East countries. The prevalence of other subepidermal autoimmune bullous diseases is relatively low. Detailed immunofl uorescence and immunoblotting studies on larger patient numbers would help better characterize the pattern of subepidermal autoimmune bullous diseases and their features in Indian patients.
RESUMO
Pemphigus is a potentially fatal autoimmune epidermal bullous disorder. Rituximab is a novel therapy for the treatment of refractory pemphigus. However, there is limited clinical data on safety and efficacy of rituximab in pediatric age group. Herein, we report an 11-year-old boy of childhood pemphigus vulgaris who failed to respond to dexamethasone pulse therapy and was subsequently treated with rituximab and achieved complete remission.
RESUMO
Background Autoimmune bullous diseases (ABD) represent a group of chronic blistering dermatoses in which management is often challenging. Epidemiologic data on these diseases in Malaysia has been limited. Objectives Our purpose was to study the spectrum of the various ABD presented to the Department of Dermatology, Ipoh Hospital, and to determine the clinico-epidemiological pattern of the 2 main ABD, namely pemphigus and bullous pemphigoid. Methodology We performed a retrospective review of records for all patients who were diagnosed with ABD confirmed by histopathology and direct immunofluorescence test in this centre between 2001 and 2005. The data were analyzed with regard to age, sex, ethnicity, subtypes of ABD, treatment provided and outcome. Results There were a total of 79 cases of ABD presented to us during this period. Bullous pemphigoid was observed to be the commonest (60.8%) followed by the pemphigus group (36.7%) with the mean incidence of 0.45/100,000/year and 0.28/100,000/year respectively.44% of patients were of ethnic Chinese origin. There was an overall female preponderance. The mean age of presentation was 65.5 years for bullous pemphigoid and 55 years for pemphigus group. The mean duration of disease before presentation was 1.6 months for bullous pemphigoid and 6.3 months for pemphigus. Various combinations of immunosuppressive agents were used to treat the patients. 48% of bullous pemphigoid cases were controlled with prednisolone alone while 67.9% of pemphigus group required at least 2 immunosuppressive agents to achieve disease control. Conclusion In our study population, bullous pemphigoid was more frequently seen than pemphigus.