Estigmas, pesquisas e embates: uma história do pênfigo brasileiro, séculos XIX e XX / Stigmas, studies, and strife: a history of Brazilian pemphigus, nineteenth and twentieth centuries

Resumo O artigo apresenta uma narrativa histórica sobre a incidência do pênfigo foliáceo no Brasil ao longo dos séculos XIX e XX. Doença bolhosa autoimune da pele que acomete com mais frequência crianças, adolescentes e adultos jovens que vivem nas áreas rurais de regiões endêmicas. Foi descrita pela primeira vez no país em 1903, pelo médico Caramuru Paes Leme. Os principais focos se situam no Distrito Federal e nos estados de Goiás, Mato Grosso, Mato Grosso do Sul, Minas Gerais, Paraná e São Paulo. Temática de pesquisa amplamente visitada pela ciência médica, em especial a dermatologia, não tem merecido a atenção peculiar por parte dos historiadores da saúde e da doença.

Abstract This paper presents a historical narrative about the incidence of pemphigus foliaceus in Brazil in the nineteenth and twentieth centuries. This autoimmune blistering skin disease is more common in children, adolescents, and young adults who live in rural areas of endemic regions. It was first described in Brazil in 1903 by the physician Caramuru Paes Leme. The main foci of the disease are in the Federal District and the states of Goiás, Mato Grosso, Mato Grosso do Sul, Minas Gerais, Paraná, and São Paulo. This research topic, which has attracted widespread attention from medical practice, especially dermatology, has not received similar attention from historians of health and disease.

Gaze vaselinada para prevenção de lesões por pressão em pessoas com dermatoses imunobolhosas / Vaseline gauze for prevention of pressure injuries in people with immunobullous dermatoses / Gasa con vaselina para la prevención de lesiones por presión en personas con dermatosis inmunobulosa

Objetivo: avaliar a mobilidade do cliente com dermatose imunobolhosa antes e após aplicação do curativo com gaze vaselinada. Método: estudo quase experimental, interinstitucional, com clientes com dermatoses imunobolhosas hospitalizados em um hospital estadual e um hospital federal do Estado do Rio de Janeiro e uma instituição do Mato Grosso do Sul. Utilizou-se a lógica fuzzy para classificar a mobilidade dos sujeitos antes, 24 horas após e uma semana após aplicação do curativo. A pesquisa foi aprovada pelo Comitê de Ética em Pesquisa. Resultados: Incluídos 14 participantes, sendo nove com pênfigo vulgar, dois com pênfigo foliáceo e três com penfigóide bolhoso, entre 27 e 82 anos, predominando 11 mulheres. Após 24 horas, nenhum participante se considerou com baixa mobilidade, sete passaram a mobilidade média, e sete, alta, o que foi mantido uma semana após aplicação do curativo. Conclusão: constatou-se significativo aumento da mobilidade logo nas primeiras 24 horas após aplicação do curativo.

Objective: to assess the mobility of clients with immunobullous dermatoses, before and after applying vaseline gauze dressings. Method: in this quasi-experimental, interinstitutional study of inpatients with immunobullous dermatoses at a state hospital and a federal hospital in Rio de Janeiro State and an institution in Mato Grosso do Sul (Brazil), patient mobility before, 24 hours after, and one week after applying the dressing was classified using fuzzy logic. The study was approved by the research ethics committee. Results: 14 participants, nine with pemphigus vulgaris, two with pemphigus foliaceus, and three with bullous pemphigoid, aged between 27 and 82 years old, and predominantly (11) women. After 24 hours, none of the participants considered their mobility to be poor, seven began to be moderately mobile, and seven were highly mobile, and continued so one week after applying the dressing. Conclusion: mobility increased significant in the first 24 hours after applying the dressing.

Objetivo: evaluar la movilidad de clientes con dermatosis inmunobullosa, antes y después de la aplicación de apósitos de gasa con vaselina. Método: en este estudio cuasi-experimental, interinstitucional de pacientes hospitalizados con dermatosis inmunobullosa en un hospital estatal y un hospital federal en el estado de Río de Janeiro y una institución en Mato Grosso do Sul (Brazil), la movilidad del paciente antes, 24 horas después y una semana después la aplicación del apósito se clasificó mediante lógica difusa. El estudio fue aprobado por el comité de ética en investigación. Resultados: se incluyeron 14 participantes, nueve con pénfigo vulgar, dos con pénfigo foliáceo y tres con penfigoide ampolloso, con edades comprendidas entre 27 y 82 años, y predominantemente mujeres (n=11). Después de 24 horas, ninguno de los participantes consideró que su movilidad fuera pobre, siete comenzaron a ser moderadamente móviles y siete eran altamente móviles, y así continuaron una semana después de la aplicación del apósito. Conclusión: la movilidad aumentó significativamente en las primeras 24 horas después de la aplicación del apósitoconsideraba con baja movilidad, siete comenzaron a tener movilidad media y siete, alta, que se mantuvo una semana después de aplicar el apósito. Conclusión: hubo un aumento significativo en la movilidad en las primeras 24 horas después de aplicar el apósito.

Afecciones perianales no infecciosas, no neoplásicas, del adulto / Noninfectious, non-neoplasic adult perianal affections

Las enfermedades perianales del adulto, de carácter no infeccioso y no neoplásico, son un motivo de consulta poco frecuente. Se caracterizan por la variedad de su etiología y de su sintomatología clínica, y plantean dificultad en el diagnóstico y en la terapéutica. El objetivo del presente trabajo es abordar una patología que plantea la necesidad de una intervención interdisciplinaria. Se incluyen consideraciones anatomopatológicas, clínicas y terapéuticas. (AU)

Noninfectious, non- neoplasic perianal affections are uncommon diseases. They are characterized by the variety of the etiology and clinical symptomatology, posing difficulty in diagnosis and therapeutics. The objective of this paper is to address a pathology that raises the need for interdisciplinary intervention anatomopathological, clinical and therapeutic considerations are included. (AU)

Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.

Pemphigus vulgaris

Abstract: Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.

Pênfigo: uma cartografia sobre as articulações das políticas em saúde / Pemphigus: cartography on the coordination of health policies

Resumo Pênfigos são um grupo de doenças bolhosas autoimunes que acometem a pele e mucosas, cuja incidência mundial é de 0,75-5/1.000.000 casos ao ano. Nossa problematização parte das relações de poder/verdade e subjetivação que produzem modalidades de experiência sob a perspectiva hospitalar e das políticas públicas. Pesquisa qualitativa cartográfica, sustentada sob uma perspectiva Deleuziana. Nossos objetivos foram problematizar o pênfigo como doença-crônica e discutir as políticas de saúde e sua repercussão nas práticas de cuidado. Percorremos esta cartografia em um hospital filantrópico de referência nacional para tratamento de pênfigo. Esta pesquisa foi construída de setembro de 2012 a fevereiro de 2013. Os materiais de análise foram: prontuários, relatos em diário de campo de todas as atividades assistenciais realizadas, tais como: consultas médicas, procedimentos técnicos realizados pela equipe de enfermagem e equipe médica e relatos de pacientes, familiares, médicos, e equipe de enfermagem. Nossas análises foram construídas sob uma perspectiva histórico-genealógica Foucaultiana. Consideramos que as políticas de saúde a doentes crônicos com pênfigo não se apresentam como uma condição a ser evitada/prevenida, mas como uma anormalidade de grupos, bioidentidades que não têm efeito no conjunto da população.

Abstract Pemphigus is a group of autoimmune blistering diseases that affect the skin and mucous membranes, the global incidence of which is 0.75 to 5 / 1.000.000 cases a year. The approach taken derives from the relations of power/truth and subjectivity that produce modes of experience from the perspective of hospitals and public policies. It involves cartographic qualitative research, supported from a Deleuzian standpoint. The objective was to discuss pemphigus as a chronic disease and discuss health policies and their impact on care practices. This cartography was conducted in a philanthropical hospital, which is a national reference for the treatment of pemphigus. The research was conducted from September 2012 to February 2013. The analysis of materials included: medical records, field diary reports of all healthcare activities, such as medical consultations, technical procedures performed by nursing staff and medical staff and reports of patients, families, physicians, and nursing staff. The analyses were created from a Foucauldian historical and genealogical perspective. The conclusion reached is that health policies for chronic patients with pemphigus are not presented as a condition to be avoided / prevented, but as abnormal groups and bio identities that have no effect on the whole population.

Pénfigo paraneoplásico asociado a leucemia linfocítica crónica / PARANEOPLASTIC PENPHIGUS ASSOCIATED TO CHRONIC LYMPHOCYTIC LEUKEMIA

El pénfigo paraneoplásico es una dermatosis ampollar autoimmune asociada a un proceso neoplásico, conocido previamente o no. Se presenta con mayor frecuencia en hombres, entre los 45 y 70 años, pero también puede manifestarse en niños. La clínica es polimorfa. Existen 5 variantes posibles: pénfigo símil, penfigoide ampollar símil, eritema multiforme símil, enfermedad de injerto contra huésped símil y liquen plano símil. Todas ellas comparten una característica en común: la estomatitis severa, dolorosa, progresiva y refractaria a los tratamientos convencionales, que obliga a descartar esta enfermedad. Si bien los antígenos involucrados en su fisiopatogenia son múltiples, la detección de anticuerpos Ig G anti envoplaquina y anti periplaquina constituye el método de diagnóstico más específico. En la actualidad se utilizan los criterios de diagnóstico de Camisa y Helm, que se basan en hallazgos clínicos (erupción mucocutánea polimorfa), histológicos (acantolisis) e inmunohistoquímicos (inmunofluorescencia directa, indirecta e inmunoprecipitación positivas). El abordaje de esta enfermedad debe considerar dos aspectos: el tratamiento del pénfigo en sí y el de la neoplasia asociada, que puede ser benigna (raro) o maligna (con mayor frecuencia). El pronóstico es severo (mortalidad del 75-90%) y en la mayoría de los casos la causa de muerte depende de las complicaciones de la enfermedad, como sepsis y bronquiolitis obliterante. Se presenta un paciente varón de 67 años con PPN asociado a Leucemia Linfocítica Crónica que respondió satisfactoriamente al tratamiento instaurado (meprednisona + quimioterapia con fludarabina, ciclofosfamida y rituximab) y no presentó recidiva a más de 2 años de seguimiento interdisciplinario.

Paraneoplastic pemphigus is an autoimmune blistering disease associated with neoplasms, previously known or not. It occurs most often in men, between 45-70 years, but can also manifest in children. The clinic is polymorphic. There are 5 possible variants: pemphigus-like, bullous pemphigoid-like, erythema multiforme-like, graft versus host disease-like and lichen planus-like. All of them share a common characteristic: a severe, painful, progressive and refractory to conventional treatments stomatitis, which forces us to rule out this disease. Although the antigens involved in its physiopathogenesis are multiple, the detection of anti-envoplakin and anti-periplakin IgG antibodies constitutes the most specific diagnostic method. At present, the diagnostic criteria of Camisa and Helm are used, which are based on clinical (polymorphic mucocutaneous eruption), histological (acantholysis) and immunohistochemical findings (direct and indirect immunofluorescence and immunoprecipitation positive). The approach to this disease must consider two aspects: treatment of the pemphigus as well as the associated neoplasm, which can be benign (rare) or malignant (more frequently). The prognosis is severe (mortality of 75-90%) and in most cases the cause of death depends on the complications of the disease, such as sepsis and bronchiolitis obliterans. We present a 67-year-old male patient with PPN associated with Chronic Lymphocytic Leukemia who responded satisfactorily to the established therapy (meprednisone + chemotherapy with fludarabine, cyclophosphamide and rituximab) and did not present recurrence after more than two years of interdisciplinary follow-up.

Oral Pemphigus Vulgaris: A Case Report.

Pemphigus vulgaris is an autoimmune mucocutaneous disorder that affects the skin and other mucous membranes of which the intra-oral lesions are first to appear. The occurrence of this disease is very few in the general population. Nevertheless, pemphigus vulgaris is a critical condition because if untreated, it often results in patient’s death. This disease is characterized by the production of autoantibodies against intercellular bridges. Most of the patients are misdiagnosed. Hence, it is necessary for the dental professionals to be aware with the clinical manifestations of this disease to ensure early diagnosis and treatment. Here, we present a case of 57-year-old female patient, which was diagnosed as pemphigus vulgaris.

Pénfigo foliáceo endémico / Endemic pemphigus foliaceus (fogo selvagem)

El pénfigo foliáceo endémico (P F E) o fuego salvaje es una dermatosis ampollar auto inmune presente en áreas de la Amazonia peruana. Se caracteriza por vesículas intraepidérmicas acantolíticas, localizadas en la región subcorneal y en cuya etiología participan anticuerpos contra desmogleína 1, una glicoproteína encargada de la unión intercelular de las células epidérmicas. Se presenta el caso de en una mujer de 52 años con PFE grave, de evolución fulminante y desenlace fatal por sepsis...

The endemic pemphigus foliaceus (EPF) or fogo selvagem is an autoimmune bullous dermatosis located in areas of our Amazon jungle .It is characterized by intraepidermic acantholytic vesicles in the subcorneal region and its etiology involves the presence of antibodies to desmoglein 1, a glycoprotein responsible for the intercellular junctions of the epidermal cells. We present the case of a 52-year-old woman with severe PFE with fulminant evolution and fatal outcome due to sepsis...

Aplicación de un Plan de Cuidados Enfermeros utilizando el modelo de Virginia Henderson (catorce necesidades) a una persona que presenta lesiones causadas por pénfigo vulgar / Implementation of a plan of care using the model of Virginia Henderson (fourteen needs), to a person who presents lesions caused by pemphigus vulgaris

Presentamos un caso clínico cuyo interés es mostrar el Plan de Atención de Cuidados Enfermeros (PLACE). La metodología guiada está basada en la taxonomía NANDA, NOC y NIC, la cual utiliza el modelo de Virginia Henderson de las catorce necesidades, esto con la finalidad de difundir la labor diaria del profesional de enfermería. En el PLACE planteamos la posibilidad también, de referirse a éste, como tratamiento enfermero en un futuro más próximo, cuyos objetivos son aplicar los conocimientos científicos fundamentados, razonados y planeados, a fin de resolver necesidades de salud centrados en la persona desde su padecimiento hasta su rehabilitación, orientados con su método principal: el Proceso de Atención de Enfermería (PAE), el cual permite aplicar cuidados a personas sanas o enfermas, pudiéndose aplicar desde una clínica de heridas y ostomías hasta para la gran diversidad de personas en distintas edades que se encuentren en servicios de hospitalización, clínica o a domicilio, así como en aquellas que presenten diferentes necesidades de atención en su salud. El caso clínico difundido es de una mujer con diagnóstico médico de pénfigo vulgar, que presenta lesiones epidérmicas en una gran superficie corporal y úlceras por presión de IV grado a nivel sacro, la cual es intervenida interdisciplinariamente en una Clínica de Heridas y Ostomías (CHO). De acuerdo con lo antes mencionado, haremos énfasis en el tratamiento de enfermería

We present a clinical case where the interest is to show a Plan of Nursing Care (PLACE) with a guided methodology based on the taxonomy NANDA, NIC and NOC using the Virginia Henderson model of the fourteen needs, with the purpose of disseminating the daily work of the nursing professional; for the purpose of disseminating the daily work of the nursing professional; in the place we have the possibility also to refer to this as treatment nurse in a near future; whose objectives are to apply sound scientific knowledge, reasoned and planned, in order to resolve health needs focusing on the person from their disease, and follow him to his rehabilitation, oriented with its main method: The process of Nursing Care (PAE), that allows you to apply care to people healthy or diseased, which could be applied from a clinic of wounds and ostomies, to the great diversity people in different ages, who are in hospitalization services, clinic or home, as well as in those who present different care needs in their health. He clinical case spread, is a female person with a medical diagnosis of pemphigus vulgaris, which presents epidermal injury in a large body surface area and pressure ulcers of level IV degree to sacrum, which is operated in an interdisciplinary way in a clinic of wounds and Ostomies (CHO). According to the described we will place emphasis on the treatment of nursing

Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. These are reviewed in this article. Future research may help definitively to locate the position of these forms in the pemphigus group, especially with regard to pemphigus herpetiformis and the IgG/ IgA pemphigus.

The frequency of osteoporosis in patients with pemphigus vulgaris on treatment.

Background: Pemphigus vulgaris was almost fatal before the advent of glucocorticoids. Unfortunately, the high doses and prolonged administration of glucocorticoids, which often needed to control the disease, result in numerous adverse effects many of which are serious. Aims: To evaluate the patients with pemphigus vulgaris on treatment in respect of osteoporosis and to compare the frequency of osteoporosis in these patients with the healthy ones. Methods: The study consisted of 40 patients with pemphigus vulgaris and 34 healthy controls. Bone mineral density measurements were obtained by dual- energy X-ray absorptiometry. Blood serum, bone parameters, and biochemical hormonal measurements were examined in both groups. Results: When the bone mineral density values of patients with pemphigus vulgaris were compared with those of the control group, there was no significant difference between hip bone mineral density values, while lumbar region T and Z scores were found significantly low in the patient group (p = 0.034 and p = 0.006, respectively). Osteoporosis, osteopenia, and normal dual-energy X-ray absorptiometry rates in the patient group were found to be 32.5%, 32.5%, and 35%, respectively. These rates were found to be 18%, 23%, and 59% in control group, respectively. There were more fractures in the patient group and the difference was statistically significant (p = 0.004). Conclusion: An increase in osteoporosis frequency and secondary fracture to osteoporosis in the patients with pemphigus vulgaris was detected.

Estudio clínico epidemiológico del pénfigo en el Hospital Nacional Edgardo Rebagliati Martins (2006-2010) / Clinical epidemiological study of pemphigus at the National Hospital Edgardo Rebagliati Martins (2006-2010)

INTRODUCCION: El pénfigo es una enfermedad ampollar autoinmune poco frecuente, que afecta a la piel y mucosas. Clásicamente se divide en dos grandes grupos, el pénfigo vulgar y pénfigo foliáceo. OBJETIVOS: Conocer datos epidemiológicos y clínicos de la población tratada en el HNERM. METODOLOGIA: Estudio descriptivo, observacional, retrospectivo. RESULTADOS: Reportamos igual número de casos de pénfigo vulgar y foliáceo (47.9 por ciento). El pénfigo vulgar afectó más a mujeres (60 por ciento) y el pénfigo foliáceo afectó más a varones (60 por ciento). La presentación inicial para el pénfigo vulgar fue cutáneo (57 por ciento). El tratamiento más usado fueron los corticoides. CONCLUSIONES: Reportamos una incidencia de 0.24 y 0.12 por 100,000 personas por año para el pénfigo vulgar y foliáceo respectivamente. Encontramos similar número de casos de pénfigo vulgar y foliáceo. El pénfigo vulgar afecta más a mujeres y el pénfigo foliáceo afecta más a varones. El promedio de edad de las personas afectadas es de 50 años. Las zonas más afectadas son la región del tórax y la cabeza. La histopatología fue concluyente en más del 50 por ciento de pacientes. Las tasas de remisión completa y parcial se dieron en más del 50 por ciento de casos. E tratamiento inicial más usado fueron los corticoides EV y la ciclofosfamida EV y en cuanto a tratamiento de mantenimiento lo más usado fueron los corticoides orales, la azatioprina y la ciclofosfamida oral. Encontramos que las infecciones y la osteoporosis son las principales complicaciones.

Endemic pemphigus in the peruvian Amazon: epidemiology and risk factors for the development of complications during treatment / Pênfigo endêmico na Amazônia peruana: epidemiologia e fatores de risco para o desenvolvimento de complicações durante o tratamento

BACKGROUND: Pemphigus is an autoimmune blistering disease. According to a report, in areas of endemic pemphigus foliaceus (EPF) in Peru there are cases of pemphigus vulgaris with epidemiologic, clinical and histopathologic characteristics similar to those of "endemic pemphigus vulgaris" (EPV) in Brazil. OBJECTIVES: To determine the clinical and epidemiologic characteristics of endemic pemphigus and the risk factors of patients for developing complications during treatment. METHODS: A study was carried out from July 2003 to March 2008. The study population was 60 patients with EPF and 7 patients with EPV evaluated in hospitals and clinics in the Peruvian Amazon and Lima. A multivariate analysis was carried out using binary logistic regression. RESULTS: The average age of EPF patients was 31.4 years; 55% were men; 60% presented the generalized clinical variant. Non-compliance with the treatment was seen in 57.1% of the patients. Thirty-five percent presented complications (e.g. pyodermitis and pyelonephritis) during treatment. The risk factors for developing complications during treatment were non-compliance with the treatment and having the generalized clinical form. In the EPV group, the average age was 21.7 years; 71.4% were men. All patients presented with the mucocutaneous clinical variant and the initial presentation consisted of oral mucosa lesions; 71.4% presented complications during treatment, pyodermitis being the most frequent. CONCLUSIONS: Non-compliance with the treatment and the generalized clinical form are risk factors for the development of complications during treatment of patients with EPF. Peru indeed has EPV cases with epidemiologic characteristics similar to EPF. Living in a rural area may represent a risk factor for the development of complications during treatment of patients with EPV.

FUNDAMENTOS: O pênfigo é uma doença auto-imune bolhosa. Segundo um relatório, em áreas de pênfigo foliáceo endêmico no Peru há casos de pênfigo vulgar com características epidemiológicas, clínicas e histopatológicas semelhantes às do "pênfigo vulgar endêmico" no Brasil. OBJETIVOS: Determinar as características clínicas e epidemiológicas do pênfigo endêmico e os fatores de risco para o desenvolvimento de complicações durante o tratamento. MÉTODOS: Um estudo foi realizado de julho de 2003 a março de 2008. 60 doentes de pênfigo foliáceo endêmico e 7 de pênfigo vulgar endêmico foram avaliados em hospitais e clínicas na Amazônia peruana e em Lima. Uma análise multivariante foi feita usando regressão logística binária. RESULTADOS: A idade média dos doentes de pênfigo foliáceo endêmico foi 31,4 anos; 55% eram homens, 60% apresentavam a forma clínica generalizada. 57,1% nao cumpriram o tratamento. 35% apresentaram complicações (por exemplo, piodermites e pielonefrite). Os fatores de risco foram não cumprir o tratamento e ter a forma clínica generalizada. No grupo pênfigo vulgar endêmico, a idade média foi 21,7 anos; 71,4% eram homens. Todos os pacientes apresentavam a variante clínica mucocutânea e a apresentação inicial consistia de lesões da mucosa bucal; 71,4% apresentaram complicações durante o tratamento, piodermites sendo a mais freqüente. CONCLUSÕES: Não cumprir o tratamento e ter a forma clínica generalizada são fatores de risco para o desenvolvimento de complicações durante o tratamento de pênfigo foliáceo endêmico. Peru realmente tem casos de pênfigo vulgar endêmico com características epidemiológicas semelhantes às do pênfigo foliáceo endêmico. Viver numa área rural pode ser um fator de risco para o desenvolvimento de complicações.

Evaluación inmunopatológica del pénfigo foliáceo endémico luego de seis meses de tratamiento: seguimiento de cinco casos de la Amazonía peruana / Evaluation of the immunopathologic response in patiens with endemic pemphigus foliaceus after six months of systemic corticosteroid therapy

Objetivo. Describir la respuesta inmunopatológica de cinco pacientes peruanos con pénfigo foliáceo endémico antes y seis meses después de la terapia con corticosteroides sistémicos. Material y métodos. Estudio descriptivo de tipo serie de casos. Los pacientes fueron sometidos a examen físico, se obtuvodatos cínicos y, luego, una muestra de sangre, a la que se le realizó inmunofluorescencia indirecta (IFI). Se usó como sustratos esófago de mono y piel humana normal, Elisa IgM antidesmogleína 1, Elisa IgG antidesmogleína 1 (total y subclases), inmunoprecipitación (IP) antidesmogleína 1 y antidesmogleína 3. Los pacientes recibieron tratamiento con corticosteroides sistémicos por seis meses obteniéndose nuevamente una muestra de sangre para estudiar su evolución inmunopatológica. Resultados. Al examen físico inicial tres pacientes presentaban la forma clínica generalizada y dos, la localizada; a los seis meses de tratamiento solo un paciente presentaba lesiones activas debido a que su tratamiento había sido irregular. La IgManti-DSG-1 no mostró variaciones importantes manteniéndose positiva en un paciente y haciéndose positiva en un paciente inicialmente negativo. El Elisa para anticuerpos IgG4 se redujo notablemente en todos los pacientes incluidos los que tenían tratamiento irregular. De los dos pacientes que presentaban anticuerpos anti-DSG-3 (Elisa) uno redujo sus valores índices y elotro se hizo negativo; mientras que un paciente inicialmente negativo desarrolló al final del tratamiento anti-DSG-3. Conclusión. La respuesta antidesmogleína 1 IgG total e IgG4 así como la respuesta antidesmogleína 3 muestran reducción de sus valores índices con tendencia a la negativización luego de seis meses de tratamiento lo cual es evidenciado también con la IFI. La respuesta IgM antidesmogleína 1 no mostró variaciones importantes a pesar del tratamiento.

Aim. To describe the immunopathologic response in five patients with endemic pemphigus foliaceus (EPF) before and after six months of therapy with systemic corticosteroids. Material and methods. Case series study. Each patient was examined by a dermatologist who gathered the clinical information. A serum sample was obtained for indirect immunofluorescence (IFI) with monkey esophagous (ME) and normal human skin (NHS) assubstrates. Also, Elisa for IgM and IgG (total and subclasses) antidesmoglein 1 antibodies and immunoprecipitation (IP) for antidesmoglein 1 and 3 were carried out. All the patients received treatment with systemic corticosteroids for six months and blood serum was drawn after wards to assess the immunopathologicresponse. Results. On physical exam, 3 patients had the generalized form ofEPF, 2 patients had the localized form and one patient had someactive lesions because he was not compliant with the therapy.Anti-IgM desmoglein 1 values were decreased in 4 patients buta couple of the values remained unchanged or even increased because they were not completely compliant with the treatment.IgG4 desmoglein 1 antibodies were notably decreased in all the patients. A couple of patients who had antibodies against desmoglein 3 decreased, being one of them negative. One of the patients was initially negative for antibodies against desmoglein3 but later on, he developed anti desmoglein 3 antibodies.Conclusion. The immunological response against IgG and IgG4desmoglein 1 as well as against desmoglein 3 were reduced after the treatment and showed a tendency to become negative. This was correlated with IFI values as well. IgM desmoglein antibodies didnot show important variations despite of the corticosteroid treatment.

Uso de anticuerpos monoclonales anti-CD20 (rituximab) en paciente con pénfigo vulgar grave / Use of monoclonal anti-CD20 (rituximab) in a patient with severe pemphigus vulgaris

El pénfigo vulgar es una enfermedad vesículo-ampollosa de carácter autoinmunitario que compromete la piel y las membranas mucosas, y presenta un curso crónico. La inmunopatogénesis se basa en la presencia de autoanticuerpos (IgG) producidos por linfocitos B y dirigidos contra la superficie de los queratinocitos, que generan pérdida de la adherencia intercelular y la formación de ampollas subepidérmicas. La morbimortalidad es variable, y es muy importante el manejo que se instaure. Se han utilizado múltiples tratamientos inmunosupresores, principalmente corticosteroides sistémicos y múltiples inmunorreguladores, sin embargo, en algunos casos resistentes o ante la presencia de efectos secundarios, la terapia inmunosupresora convencional es insuficiente para controlar la enfermedad y disminuir la morbimortalidad. Se presenta una revisión sobre el uso de anticuerpos monoclonales anti-CD20 de los linfocitos B (rituximab), para el manejo del pénfigo vulgar resistente y con mala respuesta a múltiples tratamientos convencionales a altas dosis y por periodos prolongados.

Pênfigo vulgar: relato de caso / Pemphigus vulgaris: a case report

O pênfigo é uma doença vesicobolhosa caracterizada pela presença de auto-anticorpos contra moléculas de adesão intraepidérmicas. As quatro formas mais conhecidas são: pênfigo vulgar, pênfigo vegetante, pênfigo foliáceo e o eritematoso. O pênfigo vulgar é doença órgão específica, em que auto-anticorpos IgG se dirigem contra desmogleína 3, culminando no processo de acantólise. Em grande porcentagem dos casos se manifesta inicialmente na mucosa, das quais a maisafetada é a oral. O tratamento mais utilizado é a corticoterapia oral, muitas vezes em altas doses e por períodos prolongados. Como efeitos colaterais dessa terapêutica, frequentemente ocorrem: hipertensão arterial, diabete, osteoporose, infecções e distúrbios hidroeletrolíticos. O diagnóstico é feito através da clínica, visualização das lesões e biópsia.

Pénfigo vulgar: criterios altuales / Pemphigus Vulgaris: current approaches

Se realizó una revisión bibliográfica sobre pénfigo vulgar, utilizando varias bases de datos como: LIS, MEDLINE, COCHRANE, LILACS, SeCimed; con el objetivo de actualizar la información sobre esta patología, en cuanto a: prevalencia, signos y síntomas más frecuentes, morfología, métodos diagnósticos que se emplean y tratamiento. Esta enfermedad cutáneo-mucosa, no es muy frecuente en nuestro país (0.1%-0.5%); sin embargo, es la forma más habitual y grave de los pénfigos. Se presenta con mayor frecuencia en la cuarta y sexta década de la vida, siendo extraña en niños. No tiene predilección ni por el sexo ni por la raza, su etiología no está definida, aunque se asocia con trastornos inmunológicos. Se caracteriza por la aparición de ampollas relativamente tensas, en piel y mucosas, las cuales posteriormente se rompen y forman erosiones de superficies maceradas, recubiertas con material blanquecino y muy dolorosas. En las mucosas se pueden extender a labios, faringe y laringe, dificultando la ingestión de alimentos, haciendo que los pacientes se debiliten, y junto con la pérdida de electrolitos a través de las lesiones de piel, puedan llevarlo a la muerte. Lo que motivó esta revisión fue que generalmente, se presenta primero en la boca, por lo que es de suma importancia que el estomatólogo esté familiarizado con la enfermedad, a fin de diagnosticarla y tratarla precozmente, haciendo que el tratamiento sea más sencillo y, por lo tanto, menos doloroso y molesto al paciente.

It was carried out a literature review on vulgar pemphigus, using several databases like: LIS, MEDLINE, COCHRANE, LILACS, SECIMED; with objective of modernizing information on this pathology, as for: prevalence, signs and more frequent symptoms, morphology, diagnoses methods that are used and treatment. This mucous coetaneous illness isn't very frequent in our country (0.1%-0.5%) however it's most usual and serious pemphigus variety. It's presented with more frequency between fourth and sixth decade of life, being uncommon in children. It haven't predilection neither for genre neither for race, their etiology is not defined, although it associates with immunologic dysfunctions. It's characterized by appearance of relatively tense bladders, in skin and mucous, forming erosions of macerated surfaces, whitish covered and very painful material later. In mucous ones they can extend to lips, pharynx and larynx, hindering ingestion of foods, making patients to weaken, and together electrolytes through the skin lesions, they can take it to death. What motivated this revision was that generally, it's presented first in mouth, for what is of supreme importance that deontologist is familiarized with illness, in order to diagnose it and to treat precociously, making treatment simple and therefore less painful and bother patient.