Assessment of the quality of life of Egyptian and Tunisian autoimmune bullous diseases' patients using an Arabic version of the autoimmune bullous disease quality of life and the treatment of autoimmune bullous disease quality of life questionnaires

Abstract: Background: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. Objectives: We aimed to assess the ABQOL and TABQOL in the Arabic population. Methods: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again. Results: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach's alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=-0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=-0.3, p value was 0.007. Study limitations: Small sample size of some AIBDs and patients with severe disease. Conclusion: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient's distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.

Urticaria papular y sus agentes causales en Colombia / Papular urticaria: A review of causal agents in Colombia

Resumen La urticaria papular es una enfermedad alérgica causada por la picadura de insectos, la cual predomina en el trópico. El objetivo de esta revisión fue profundizar en sus aspectos epidemiológicos e inmunológicos, particularmente con base en datos publicados en Latinoamérica. Se hizo una revisión no sistemática mediante la búsqueda electrónica de artículos sobre la epidemiología de la urticaria papular, las características entomológicas de los agentes causales y los mecanismos inmunológicos asociados. Según los diversos reportes de centros médicos de Latinoamérica la urticaria papular es frecuente; el único estudio de prevalencia publicado indica que afecta a una cuarta parte de los niños escolares de Bogotá. Hay información sobre la relación causal entre la exposición domiciliaria a la pulga, la pobreza y la urticaria papular en Bogotá, una ciudad representativa de las altitudes andinas. No hay estudios que indaguen directamente sobre los insectos causales en zonas cálidas, aunque se sospecha clínicamente de los mosquitos Aedes aegypti y Culex quinquefasciatus. En cuanto a su patogenia, se destaca la participación de mecanismos celulares que involucran las células colaboradoras Th2, lo cual explica que sea una condición de hipersensibilidad retardada. El papel de la inmunoglobulina E (IgE) en la urticaria papular no está tan claro. Se desconocen los antígenos derivados de los insectos que causan la enfermedad, aunque se plantea que existen moléculas comunes de reacción cruzada entre los insectos, tales como el alérgeno Cte f 2 en la pulga, y sus homólogos en los mosquitos. La urticaria papular es una condición frecuente en Latinoamérica que debe investigarse en profundidad. La caracterización inmunológica de los componentes moleculares que causan esta condición puede resolver interrogantes sobre su etiología y su patogenia.

Abstract Papular urticaria is a chronic allergic reaction induced by insect bites, which is common in the tropics. The objective of this review was to deepen on epidemiological and immunological aspects of this disease, focused on data published in Latin American countries. We conducted a non-systematic review of the literature through electronic search on the epidemiology of papular urticaria, the entomological characteristics of the causative agents and associated immunological mechanisms. Several reports from medical centers suggest that papular urticaria is common in Latin America. Only one epidemiological survey designed to estimate prevalence of papular urticaria has been published, reporting that about a quarter of children under six years of age is affected by this condition in Bogotá. There is evidence on the causal relationship among exposure to indoor fleas, poverty and papular urticaria in Bogotá, a representative city of the Andean altitudes. Information about causal insects in tropical warmer areas is scarce, although from clinical reports Aedes aegypti and Culex quienquefasciatus appear to be the most common. Th2 cellular-mediated mechanisms are involved in its pathogenesis, which explains its delayed hypersensitivity. The role of immunoglobulin E is not clear in this disease. Insect-derived antigens directly involved in papular urticaria etiology are unknown. However, it is possible that common molecules among causal insects mediate cross-reactive reactions, such as Cte f 2 allergen, found in cat fleas, and its counterparts in mosquitoes. Papular urticaria is a frequent disease in Latin America that should be further investigated. Immunological characterization of the molecular components that cause this condition may solve questions about its pathogenesis.

Nursing diagnoses in patients with immune-bullous dermatosis / Diagnósticos de enfermagem em clientes com dermatoses imunobolhosas / Diagnósticos de enfermería en pacientes con inmuno-dermatosis ampollosa

ABSTRACT Objective: identify nursing diagnoses in patients with immune-bullous dermatosis. Method: a quantitative and descriptive research, carried out in three institutions located in Rio de Janeiro and Mato Grosso do Sul, Brazil, using the Client Assessment Protocol in Dermatology during a nursing consultation. Simple descriptive statistics was used for data analysis. Results: 14 subjects participated in the study, nine with a diagnosis of pemphigus vulgaris, pemphigus two and three of bullous pemphigoid. The age ranged between 27 and 82 years, predominantly females (11). 14 nursing diagnoses were discussed and identified from a clinical rationale in all study participants, representing the most common human responses in this sample. The application of the Assessment Protocol in Dermatology facilitated the comprehensive assessment, in addition to providing the identification of diagnostics according to the North American Nursing Diagnosis Association International. Conclusion: the nursing diagnoses presented confirm the necessity of interdisciplinary work during the care for this clientele. For better description of the phenomena related to the client in question, it is suggested the inclusion of two risk factors related in three diagnoses of this taxonomy. It is worth noting the contribution of the findings for the care, education and research in nursing in dermatology.

Resumo Objetivo: identificar diagnósticos de enfermagem em clientes com dermatoses imunobolhosas. Método: pesquisa quantitativa e descritiva, realizada em três instituições localizadas no Rio de Janeiro e no Mato Grosso do Sul-Brasil, aplicando o Protocolo de Avaliação do Cliente em Dermatologia, durante consulta de enfermagem. Utilizou-se a estatística descritiva simples para análise dos dados. Resultados: participaram do estudo 14 sujeitos, nove com diagnóstico médico de pênfigo vulgar, dois de foliáceo e três de penfigoide bolhoso. A idade variou entre 27 e 82 anos, predominando 11 pessoas do sexo feminino. Foram discutidos 14 diagnósticos de enfermagem identificados a partir do raciocínio clínico, em todos os participantes do estudo, representando as respostas humanas mais frequentes nesta amostra. A aplicação do Protocolo de Avaliação do Cliente em Dermatologia facilitou a avaliação integral, além de propiciar a identificação dos diagnósticos de acordo com a North American Nursing Diagnosis Association International. Conclusão: os diagnósticos de enfermagem apresentados ratificam a necessidade do trabalho interdisciplinar durante atendimento a esta clientela. Para melhor descrição dos fenômenos relacionados à clientela em questão, sugere-se a inclusão de dois fatores de risco/relacionados em três diagnósticos desta taxonomia. Cabe ressaltar a contribuição dos achados para o cuidar/educar/pesquisar em enfermagem em dermatologia.

RESUMEN Objetivo: identificar los diagnósticos de enfermería en pacientes con inmuno dermatosis ampollosa. Método: investigación cuantitativa y descriptiva, realizada en tres instituciones ubicadas en Río de Janeiro y Mato Grosso do Sul, Brasil, utilizando el Protocolo de Evaluación del Cliente en Dermatología en la consulta de enfermería. Se utilizó estadística descriptiva simples para el análisis de datos. Resultados: 14 sujetos participaron en el estudio, nueve con diagnóstico de pénfigo vulgar, dos de pénfigo foliáceo y tres de penfigoide ampolloso. La edad osciló entre 27 y 82 años, predominio femenino con 11 mujeres. Se discutieron 14 diagnósticos de enfermería identificados desde el razonamiento clínico, en todos los participantes en el estudio, que representa las respuestas humanas más comunes en esta muestra. La aplicación del Protocolo de Evaluación de Dermatología facilitó la evaluación global, además de proporcionar la identificación de los diagnósticos de acuerdo con la North American Nursing Diagnosis Association International. Conclusión: los diagnósticos de enfermería presentados confirman la necesidad del trabajo interdisciplinario en el servicio a estos clientes. Para una mejor descripción de los fenómenos relacionados con los clientes en cuestión, se sugiere la inclusión de dos factores de riesgo/relacionados en tres diagnósticos de esta taxonomía. Vale la pena señalar la contribución de los hallazgos para el cuidado/educación/investigación en enfermería en dermatología.

Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience

BACKGROUND: Immunofluorescence testing is an important tool for diagnosing blistering diseases. OBJECTIVE: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases. METHODS: We retrospectively analyzed immunofluorescence results encompassing a 10-year period. RESULTS: 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate. CONCLUSION: Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect ...

Enfermedades ampollosas autoinmunes del grupo de los pénfigos / Autoimmune blistering diseases of the pemphigus group

En muchas enfermedades dermatológicas se presentan ampollas, pero no todas son de etiología autoinmune. Para el estudio de las enfermedades ampollosas se deben tener en cuenta las manifestaciones clínicas, la historia de cómo y cuándo empezaron las ampollas, las características epidemiológicas e histológicas (por ejemplo, el nivel de la piel en el que se producen las ampollas) y la presencia o no de infiltrados inflamatorios. Para corroborar la etiología autoinmune de la enfermedad ampollosa es importante contar con los resultados de pruebas como la inmunofluorescencia directa e indirecta, el inmunoblotting, el ensayo inmunoenzimático (ELISA), la inmunoprecipitación y la microscopía electrónica. La información sobre los títulos séricos de autoanticuerpos ayuda a orientar mejor el tratamiento inmunosupresor.

Blisters may appear in many dermatological diseases, but they are not necessarily of autoimmune etiology. For the study of blistering diseases, it is necessary to take into account the clinical aspects, the history of when and how blisters appeared, the epidemiological and histological information (for instance, the skin level at which blisters are located), and whether inflammatory infiltrates are present. In order to corroborate the autoimmune etiology of blisters, it is important to have the results of confirmatory tests such as direct and indirect immunofluorescence, immune blotting, enzyme-linked immune-assay (ELISA), immune precipitation, and electronic microscopy. Information on autoantibodies serum titers may help to conduct a more precise immunosuppressive therapy.

Expresión de IL-10, IL-4 e IFN- γ en lesiones activas de piel en niños con urticaria papular por picadura de pulga / Expression of IL-10, IL-4 and IFN-γ in active skin lesions of children with papular urticaria

Introducción. La urticaria papular por picadura de pulga se conoce como una enfermedad alérgica. Sin embargo, las investigaciones no muestran una clara relación con las enfermedades alérgicas. Objetivo. Estudiar la expresión de IL-10, IL-4 e IFN-γ, como marcadores de la respuesta efectora de células T en lesiones de piel de pacientes con urticaria papular por picadura de pulga. Materiales y métodos. Se incluyeron 14 biopsias de lesiones de piel de niños con diagnóstico de urticaria papular por picadura de pulga y 5 biopsias de piel sana obtenidas de niños sometidos a cirugía por enfermedades no inflamatorias. Todas las muestras se obtuvieron de niños menores de 12 años. Se extrajo ARN con trizol y se cuantificaron los niveles de expresión de las citocinas con la técnica de reacción en cadena de la polimerasa en tiempo real. Resultados. En los pacientes con urticaria papular por picadura de pulga, se encontró amplia diversidad en los niveles de expresión de IFN-γ e IL-10, y valores bajos constantes para IL-4. Se observaron tres perfiles que no corresponden a un patrón común en los pacientes. Las muestras obtenidas de tejidos sanos no presentaron expresión de las citocinas. Conclusiones. Los datos corresponden a la primera descripción de citocinas que median la respuesta inmunitaria en el sitio de la lesión cutánea en niños con con urticaria papular por picadura de pulga, lo cual indica que la respuesta local es mixta ya que no se encuentra predominio de un fenotipo específico en ninguno de los pacientes.

Introduction: Papular urticaria caused by the bites of fleas traditionally has been defined as a chronic allergic disease. However, currently no clear relationship has been described between this pathology and common allergic diseases. Objective: The expression of IL-10, IL-4 and IFN-γ as markers of effector T cell responses was examined in skin lesions of patients with papular urticaria by flea bite. Materials and methods: Fourteen skin lesion biopsies were sampled from children with a clinical diagnosis of papular urticaria by flea bite and were compared with 5 healthy skin biopsies of children with no history of the disease. All children were under 12 years old. RNA was extracted with trizol and the expression levels of cytokines were analyzed by real time PCR technique. Results: A wide range in the expression levels of IFN-γ and IL-10 was noted as well as constant low values of IL-4. Three distinct profiles were observed, but which did not correspond to a recognizable pattern among the patients. The samples obtained from healthy tissues showed no expression of any of the cytokines. Conclusions: This is the first characterization of cytokines that mediate the immune response at the site of the skin lesion in children with papular urticaria by flea bite. The data indicated that the local response was mixed and that a single phenotype is not predominant among the patients.

Dermatose por IgA e IgG linear: relato de caso com boa resposta terapêutica à dapsona e ao micofenolato mofetil / Linear IgA/IgG bullous dermatosis: successful treatment with dapsone and mycophenolate mofetil

Relata-se o caso de paciente feminina, de 21 anos, com dermatose por IgA e IgG linear. Inicialmente, a resposta clínica foi favorável à dapsona. Após a interrupção desta medicação, por crise de anemia sintomática, precipitada por malária, houve piora da doença, apesar da utilização da prednisona e pulsoterapia com metilprednisolona. A reintrodução da dapsona, associada ao micofenolato mofetil, possibilitou o controle da enfermidade.

A 21-year-old female presenting linear IgA and IgG disease initially responded well to dapsone therapy. However, the treatment with dapsone was withdrawn due to severe anemia induced by malaria, which led to worsening of the clinical picture. Although prednisone and methylprednisolone were tried, the patient responded only to the association of dapsone and mycophenolate mofetil.

Dermatoses bolhosas auto-imunes / Autoimmune bullous dermatoses

Dermatoses bolhosas autoimunes são doenças cuja manifestação cutânea primária e fundamental consiste em vesículas e bolhas. Classificam-se conforme a localização da bolha, em intraepidérmica e subepidérmica. Os pacientes produzem autoanticorpos contra estruturas específicas da pele detectáveis por técnicas de imunofluorescência, immunobloting e Elisa. Os recentes avanços da biologia molecular e celular têm permitido conhecer esses autoantígenos, contra os quais os pacientes se sensibilizam e que estão localizados na epiderme ou na junção dermoepidérmica. São doenças de baixa incidência, porém de elevada morbidade e por vezes letais. O objetivo deste trabalho é revisar e descrever os progressos nos conhecimentos de quatro doenças vésico-bolhosas autoimunes: pênfigo foliáceo endêmico (fogo selvagem), pênfigo vulgar, penfigóide bolhoso e dermatite herpetiforme.

Autoimmune bullous dermatoses are diseases in which blisters and vesicles are the primary and fundamental types of skin lesion. Their classification is based on the location of the blister: intraepidermal and subepidermal. Patients produce autoantibodies against self-specific structures of the skin detectable by immunofluorescence techniques, immunoblotting and ELISA. Recent advances in molecular and cellular biology have brought to knowledge these self-antigens, against which patients are sensitized, and which are found in epidermis or in the dermo-epidermal junction. These are low incidence, but high morbidity diseases that may be fatal. The aim of this article is to review and describe the progress of four autoimmune vesiculobullous disorders: endemic pemphigus foliaceous (wild fire), pemphigus vulgaris, bullous pemphigoid and dermatitis herpetiformis.

Imunofluorescência direta de pele e mucosas: análise comparativa, clínica e histológica, de 180 exames / Direct immunofluorescence of skin and mucous membrane: clinical and histological correlation in 180 patients

O exame de imunofluorescência direta (IFD) é recurso propedêutico adicional no diagnóstico diferencial das doenças cutâneas bolhosas, auto-imunes e näo-imune-mediadas. Nós revimos os resultados de 180 exames de IFD de pele e mucosas, de pacientes atendidos no Hospital das Clínicas da Universidade de Campinas, as biopsias correspondentes, e os comparamos às observaçöes clínicas. As dermatoses foram assim classificadas: 1) bolhosas acantolíticas (n=38); 2) bolhosas subepidérmicas (n=39); 3) inflamatórias näo-bolhosas - a) colagenoses (n=53), b) outras (n=32); 4) vasculites (n=10); 5) miscelânea (n=8). Os achados clínicos e laboratoriais, a biopsia, a evoluçäo e a resposta terapêutica foram analisados, e o papel desempenhado pela IFD foi assim classificado: 1) definiu o diagnóstico; 2) complementou o diagnóstico estabelecido; 3) näo contribuiu para o diagnóstico. Virtualmente todos os casos de pênfigo mostravam-se positivos. Neles, a IFD, unicamente, auxiliou a excluir os poucos diagnósticos diferenciais formulados. Quanto às dermatoses bolhosas subepidérmicas, a IFD foi crucial. Seu emprego foi crítico em quatro pacientes com lúpus bolhoso, pois as lesöes cutâneas precederam as manifestaçöes sistêmicas e a doença revestiu-se de marcada gravidade. A IFD foi negativa em 39 por cento dos pacientes com lúpus eritematoso sistêmico e em 53 por cento daqueles com lúpus eritematoso discóide, particularmente quando sob tratamento. Nas demais colagenoses, e nas vasculites, a IFD da pele näo contribui com qualquer elemento de valor para os demais parâmetros. Concluindo, a técnica mostrou-se essencial na investigaçäo das doenças auto-imunes e bolhosas cutâneas. A otimizaçäo da sua aplicaçäo pode ser alcançada num trabalho e cooperaçäo entre os setores envolvidos, minimizando os fatores geradores de erro