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.

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.

Fogo selvagem: endemic pemphigus foliaceus

Abstract: Fogo selvagem or endemic pemphigus foliaceus is an autoimmune acantholytic anti-cadherin bullous disease that primarily affects seborrheic areas, which might disseminate. Brazil has the world's largest number of patients, mainly in the Central-West region, but the disease has also been reported in other South American countries. It affects young people and adults who have been exposed to rural areas, with occurrence of familial cases. Anti-desmoglein-1 autoantibodies are directed against desmosomal structures, with loss of adhesion of the upper layers of the epidermis, causing superficial blisters. The etiology is multifactorial and includes genetic, immune, and environmental factors, highlighting hematophagous insect bites; drug-related factors are occasionally involved. Flaccid blisters readily rupture to yield erosive-crusty lesions that sometimes resemble seborrheic dermatitis, actinic keratosis, and chronic cutaneous lupus erythematosus. The clinical presentation varies from localized to disseminated lesions. Clinical suspicion should be confirmed with histopathological and immunofluorescence tests, among others. The progression is usually chronic, and therapy varies according to clinical presentation, but generally requires systemic corticosteroid therapy associated with adjuvant immunosuppressive treatment to decrease the adverse effects of corticosteroids. Once the disease is under control, many patients remain stable on low-dose medication, and a significant proportion achieve remission.

Prevalence of Metabolic Syndrome and its components in a Brazilian sample of pemphigus patients

BACKGROUND: Pemphigus foliaceus and pemphigus vulgaris are endemic in the northeastern region of São Paulo State, Brazil. They are treated mainly with systemic corticosteroids, which may provoke osteoporosis; atherosclerosis, higher blood pressure, insulin resistance, glucose intolerance, hyperlipidemia and abdominal obesity. These side effects of corticoids also constitute criteria for the diagnosis of metabolic syndrome. OBJECTIVE: The prevalence of metabolic syndrome and each component of metabolic syndrome in Pemphigus foliaceus and pemphigus vulgaris groups was compared with Brazilian casuistic samples. METHODS: Data of 147 patients (pemphigus foliaceus 48.9% and pemphigus vulgaris 51.1%) were compiled from medical records regarding metabolic syndrome and its components, and included in the analysis. RESULTS: There was no significant difference regarding the prevalence of metabolic syndrome in pemphigus groups compared with the Brazilian casuistic samples. The analysis of each component of metabolic syndrome showed a higher prevalence of: higher blood pressure in male subjects with pemphigus vulgaris, and in pemphigus foliaceus in both genders; diabetes mellitus in both genders for pemphigus vulgaris and pemphigus foliaceus; obesity in females for pemphigus vulgaris and pemphigus foliaceus, and hypertriglyceridemia in both genders for pemphigus vulgaris and pemphigus foliaceus groups that were statistically significant compared to the Brazilian reports. Furthermore, the study noted a higher incidence of cardiovascular events in both genders in pemphigus foliaceus and pemphigus vulgaris groups than in Brazilian casuistic samples. CONCLUSION: The components of metabolic syndrome are more numerous in pemphigus when compared with Brazilian casuistic samples. Future studies are necessary to assure that metabolic syndrome may be associated with pemphigus per se, including a greater casuistic sample of patients who have not ...

Evaluation of cases of pemphigus vulgaris and pemphigus foliaceus from a reference service in Pará state, Brazil

BACKGROUND: Pemphigusis a bullous, rare and chronic autoimmune disease. There are two major forms of pemphigus: vulgaris and foliaceus. Epidemiological data and clinical outcome in patients diagnosed in the Brazilian Amazon states are still rare. OBJECTIVES: To study the occurrence of the disease during the study period and analyze the epidemiological profile of patients, the most common subtype of pemphigus, and the clinical evolution of patients. METHODS: Retrospective analysis of medical records of hospitalized patients with pemphigus foliaceus and pemphigus vulgaris in the period from 2003 to 2010 in Dermatology Service of Hospital Fundação Santa Casa de Misericórdia do Pará, Belém, Northern Brazil. RESULTS: We found a total of 20 cases of pemphigus during the study period, 8 of which were of foliaceus pemphigus and 12 of vulgaris pemphigus. Pemphigus foliaceus had the predominance of male patients (75%), showed satisfactory clinical evolution, and was characterized by absence of pediatric cases. Pemphigus vulgaris affected more women (66.7%), showed mean hospital stay of 1 to 3 months (50%), and there were three cases of death (25%). The prescribed immunosuppressive drugs included prednisone with or without combination of azathioprine and/or dapsone. Sepsis was associated with 100% of the deaths. CONCLUSIONS: The occurrence of the disease is rare, there are no familiar/endemic outbreaks in the sample. Evolution is usually favorable, but secondary infection is associated with worse prognosis. The choice of best drugs to treat pemphigus remains controversial. .

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.

Incidência do pênfigo vulgar ultrapassa a do pênfigo foliáceo em região endêmica para pênfigo foliáceo: análise de série histórica de 21 anos / Incidence of pemphigus vulgaris exceeds that of pemphigus foliaceus in a region where pemphigus foliaceus is endemic: analysis of a 21-year historical series

FUNDAMENTO: Há dois tipos principais de pênfigo: pênfigo vulgar e pênfigo foliáceo. Nos últimos anos, mudanças clínicas e epidemiológicas relacionadas aos pênfigos têm sido observadas. OBJETIVOS: Teve-se por objetivo analisar uma série histórica de 21 anos de casos de pênfigo vulgar e pênfigo foliáceo no nordeste do estado de São Paulo, área endêmica para o pênfigo foliáceo. MÉTODOS: Neste estudo descritivo, foram analisados os dados relacionados à incidência anual e à idade de início do quadro clínico compatível com pênfigo vulgar ou pênfigo foliáceo, no período de 1988 a 2008, comparando-se ambas as formas de pênfigo. RESULTADOS: O conjunto dos resultados abrange um período de 21 anos, com 103 casos de pênfigo vulgar e 163 casos de pênfigo foliáceo. A comparação das linhas de tendência em relação à incidência mostrou ser esta decrescente para o pênfigo foliáceo em comparação àquela de crescimento para o pênfigo vulgar. Houve variação ampla nas faixas de idade, com persistência da faixa mínima de 10 a 20 anos para o pênfigo foliáceo (média de idade de 32,1 anos), e clara tendência de diminuição da idade mínima para o pênfigo vulgar (média de idade de 41,5 anos), principalmente a partir da metade da primeira década do período total analisado. CONCLUSÕES: A incidência do pênfigo vulgar ultrapassa aquela do pênfigo foliáceo a partir de 1998, permanecendo assim até os dias de hoje. Esta série histórica de 21 anos vem consubstanciar a modificação da epidemiologia dos pênfigos no Brasil, suscitando novas hipóteses para a sua etiopatogênese.

BACKGROUND: There are two main clinical subsets of pemphigus: pemphigus vulgaris and pemphigus foliaceus. Clinical and epidemiological changes related to both types of pemphigus have been observed in the last years. OBJECTIVES: To analyze a 21-year historical case series of pemphigus vulgaris and pemphigus foliaceus in the northeast region of the state of Sao Paulo, where pemphigus foliaceus is endemic. METHODS: In this descriptive study, data related to annual incidence and age of onset of symptoms compatible with pemphigus vulgaris or pemphigus foliaceus were analyzed, comparing both forms, in the period from 1988 to 2008. RESULTS: The overall results cover a period of 21 years, with 103 cases of pemphigus vulgaris and 163 cases of pemphigus foliaceus. An evaluation of the trend lines regarding incidence has shown that pemphigus foliaceus is decreasing while pemphigus vulgaris is increasing. There was great variation in the age ranges, with persistence of the minimum range, from 10 to 20 years old, for pemphigus foliaceus (mean age = 32.1 years old), and clear downward in the minimum age for pemphigus vulgaris (mean age = 41.5 years old), especially from the middle of the first decade of the total period studied. CONCLUSION: The incidence of pemphigus vulgaris has been exceeding that of pemphigus foliaceus since 1998. The results of this case series comprehending 21 years corroborate the change in the epidemiology of both clinical forms of pemphigus in Brazil, raising new hypotheses for their etiology and pathogenesis.

Características epidemiológicas, clínicas y supervivencia de pacientes con diagnóstico de pénfigo atendidos en el Hospital Regional Docente de Trujillo. Período 1994-2006 / Clinical and pathological characteristic and survival of patients with diagnosis of pemphigus attended at Hospital Regional Docente de Trujillo 1994-2006

Objetivo. Determinar las características epidemiológicas, clínicas y supervivencia de los pacientes con diagnóstico de pénfigo atendidos en el Hospital Regional Docente de Trujillo (HRDT) durante el período 1994-2006. Material y métodos. Estudio longitudinal. La población estuvo constituida por pacientes con diagnóstico clínico y/o histopatológico de pénfigo atendidos en el HRDT durante el período 1994-2006. Los pacientes fueron entrevistados a través de una visita domiciliaria o mediante vía telefónica obteniéndose su edad, sexo, procedencia, ocupación, tipo de pénfigo, recaídas, tratamiento farmacológico, inicio y tiempo tratamiento. La supervivencia fue estimada utilizando el método Kaplan-Meier; mientras que para comparar las curvas de supervivencia se empleó la prueba de log-rank. Resultados. Se entrevistó en total a 33pacientes. La supervivencia global estimada a intervalos de 2, 4, 6, 8, 10 y más de 10 años tras el diagnóstico de pénfigo fué de 100,0%, 93,9%, 80,6%, 76,0%, 68,4% y 61,5%, respectivamente. La prueba de log-rank mostró que la supervivencia fue significativamente mayor en los grupos de edad de 30 a 49 años y de 50 a 69 años (p < 0,001) así como en los pacientes que iniciaron tratamiento antes de los 6 meses de enfermedad (p < 0,001). No se observó diferencias significativas entre géneros, procedencia, tipo de pénfigo, tratamiento y tiempo de tratamiento. Conclusiones. La supervivencia de los pacientescon pénfigo del HRDT a los 10 años de enfermedad fue del 68.4% siendo significativamente mayor en los grupos de edad de30 a 49 años y de 50 a 69 años como en los que iniciaron tratamiento antes de los seis meses de enfermedad.

Objective. Determine the clinical and pathological characteristic of patients with diagnosis of pemphigus attended at Hospital Regional Docente de Trujillo (HRDT) during the period 1994-2006. Material and Methods. Longitudinal study. Population was patients with clinical and/or histopathological diagnosis of pemphigus attended in the HRDT. Patients were interviewed in a home visit or by telephone, obtaining dates as age, gender, origin, occupation, type of pemphigus, relapse, pharmacological treatment, beginning and time of treatment. Survival was estimated using the Kaplan-Meier method; while the log-rank test was used for compraing the survival curves. Results. 33 patients were interviewed. The total survival estimated in intervals of 2, 4, 6, 8,10 and more than 10 years after the diagnosis of pemphigus was 100,0%, 93,9%, 80,6%, 76,0%, 68,4% and 61,5% , respectively. The log-rank test showed that the survival was significantly more in the groups aged between 30- 49 year old and in 50-69 year old(p < 0,001) as well as in patients that began their treatment beforethe 6 months of disease (p > 0,001). Significantly differences between gender, origin, type of pemphigus, treatment and time of treatment there were no found. Conclusions. Survival of patients with pemphigus at HRDT at 10 years of disease was 68,4%, being significantly more in the groups of ages between 30-49 years and 50-69 years; as well as in patients that began their treatment before the 6 months of disease.

Estudo epidemiológico das doenças dermatológicas imunologicamente mediadas na cavidade oral / An epidemiological study of immune-mediated skin diseases affecting the oral cavity

FUNDAMENTO: As doenças dermatológicas imunologicamente mediadas compõem diversas patologias que apresentam formas variadas de manifestação no organismo. OBJETIVO: Foi proposição desta pesquisa, estabelecer a prevalência das principais doenças dermatológicas imunologicamente mediadas que apresentam manifestação oral. MÉTODOS: Foram avaliados laudos histopatológicos de 10.292 casos arquivados no Serviço de Anatomia Patológica da Disciplina de Patologia Oral da Universidade Federal do Rio Grande do Norte, no período de 1988 a 2009. Dos casos diagnosticados como algum tipo de doença em estudo, coletaram-se dados clínicos como sexo, idade, raça, sítio anatômico e sintomatologia das doenças. RESULTADOS: Do total de casos registrados, no serviço supracitado, 82 (0,8 por cento) corresponderam a doenças dermato lógicas imunologicamente mediadas com manifestação na cavidade oral. As doenças encontradas neste estudo foram: líquen plano oral, pênfigo vulgar e penfigoide benigno das membranas mucosas, sendo o líquen plano oral a lesão mais prevalente, representando 68,05 por cento dos casos analisados, dos quais 64,3 por cento apresentavam-se em mu lheres, sendo a mucosa jugal o sítio anatômico mais acometido (46,8 por cento). CONCLUSÃO: A ocorrência de doenças dermatológicas imunologicamente mediadas que apresentam manifestação oral ainda é um fato incomum, semelhante ao observado na maioria das regiões mundiais. No entanto, a busca pelo diagnóstico precoce é um requisito essencial para a condução do tratamento dessas doenças, tendo em vista o possível comprometimento sistêmico do organismo nos pacientes.

BACKGROUND: Immune-mediated skin diseases encompass a variety of pathologies that present in different forms in the body. OBJECTIVE: The objective of this study was to establish the prevalence of the principal immune-mediated skin diseases affecting the oral cavity. METHODS: A total of 10,292 histopathology reports stored in the archives of the Anatomical Pathology Laboratory, Department of Oral Pathology, Federal University of Rio Grande do Norte, covering the period from 1988 to 2009, were evaluated. For the cases diagnosed with some type of disease relevant to the study, clinical data such as the gender, age and ethnicity of the patient, the anatomical site of the disease and its symptomatology were collected. RESULTS: Of all the cases registered at the above-mentioned service, 82 (0.8 percent) corresponded to immune-media ted skin diseases with symptoms affecting the oral cavity. The diseases found in this study were: oral lichen planus, pemphigus vulgaris and benign mucous membrane pemphigoid. Oral lichen planus was the most common lesion, comprising 68.05 percent of the cases analyzed. Of these cases, 64.3 percent were women and the cheek mucosa was the anatomical site most commonly affected (46.8 percent). CONCLUSION: Immune-mediated skin diseases affecting the oral cavity continue to be rare, the prevalence found in this study being similar to that reported for the majority of regions worldwide. Nevertheless, early diagnosis is indispensable in the treatment of these diseases, bearing in mind that systemic involvement is possible in these patients.

Pénfigo vulgar. Casuística del Servicio de Dermatología del Hospital Nacional / Pemphigus vulgaris. Casuistry of the Dermatology Service of the National Hospital

Introducción: El pénfigo vulgar es una enfermedad autoinmune caracterizada por ampollas suprabasales y autoanticuerpos IgG dirigidos contra la desmogleina 3 del desmosoma. El objetivo del trabajo es comunicar la casuística del Servicio de Dermatología del Hospital Nacional en un periodo de 20 años y determinar las características demográficas, clínicas y evolutivas. Material y Método: Estudio retrospectivo, observacional, descriptivo. Se incluyen pacientes de ambos sexos, de todas las edades, que son atendidos en el ambulatorio de Dermatología o en las salas de internación del Hospital y que tienen confirmación histológica. Resultados: En el periodo 1991-2011 se diagnosticaron 22 casos de pénfigo vulgar representando el 12,7% de los casos de pénfigo observados en el servicio. Predominó en el sexo femenino (13 casos), en el grupo etario de 30 a 49 años (15 casos), siendo la media de 42,1 años. La afectación de la mucosa oral se presentó en 18 casos y en 12 fue la inicial. El compromiso cutáneo observado en todos los casos, fue diseminado o generalizado (20 casos). Dos fueron de la variedad vegetante. El tratamiento más utilizado fue la prednisona sola (8 casos) o asociada con azatioprina (8 casos). La evolución fue favorable en 19 casos con curación o remisión. Dos fueron al óbito y uno no recibió tratamiento. Conclusión: El pénfigo vulgar en nuestro servicio es una patología poco frecuente, pero con importante morbimortalidad. Es el segundo tipo en frecuencia, siendo el foliáceo el más observado.

Introduction: Pemphigus vulgaris is an autoimmune disease characterized by suprabasal blisters and IgG auto antibodies directed against desmoglein 3 of the desmosome. The aim of this work is to communicate the casuistics of the Dermatology Service of the National Hospital over a period of 20 years and determine the demographic, clinical and developmental characteristics. Material and Methods: Retrospective, observational, descriptive study. It includes patients of both sexes, all ages, in treatment at the dermatology outpatient or inpatient wards of the Hospital that have histological confirmation. Results: In the period 1991-2011, 22 cases of pemphigus vulgaris were diagnosed, representing 12.7% of cases of pemphigus observed in our service. Predominance in female sex (13 cases) and in the age group 30 to 49 years (15 cases) was observed, with a mean age of 42.1 years. Oral mucosal involvement was present in 18 cases, in 12 of which it was the initial symptom. Cutaneous involvement observed in all cases was disseminated or generalized (20 cases). Two cases were of the vegetative variety. The most commonly used treatment was prednisone alone (8 cases) or associated with azathioprine (8 cases). The outcome was favorable in 19 cases, with cure or remission. Two cases ended in death and one received no treatment. Conclusions: Pemphigus vulgaris is a rare condition in our service, but with significant morbidity and mortality. It is second in frequency after the foliaceous type.

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 en un hospital general de Lima: experiencia de 10 años (1998-2007) / Pemphigus in a tertiary health care hospital of Lima: A 10-year experience (1998-2007)

OBJETIVO: Determinar las características clínicas, epidemiológicas y de laboratorio de los pacientes con pénfigo en el Hospital Nacional Dos de Mayo (HNDM), periodo 1998- 2007. MATERIAL Y MÉTODOS: Estudio descriptivo, retrospectivo y observacional. La población estuvo conformada por los pacientes con diagnóstico de pénfigo foliáceo (PF), vulgar (PV) y paraneoplásico (PP) en el HNDM durante 1998- 2007. Se revisó las historias clínicas de los pacientes obteniéndose los datos epidemiológicos clínicos y de laboratorio los cuales fueron registrados en un instrumento de recolección de datos (validado mediante juicio de expertos). RESULTADOS: Se estudió 38 casos de pénfigo, de los cuales 29 correspondieron a PF, 8 a PV y 1 a PP. De los 29 pacientes con PF, 22 (75.9 por ciento) correspondieron a la forma no endémica y los 7 restantes a la forma endémica (24.1 por ciento). Con respecto a la evolución, el 27.3 por ciento de los pacientes presentaron remisión de su enfermedad y las complicaciones más frecuentes fueron la piodermitis (24.1 por ciento), neumonía (17.2 por ciento) y necrosis aséptica de cadera (17.2 por ciento). De los 8 pacientes diagnosticados con PV, 2 de ellos (25.0 por ciento) correspondieron a la forma endémica, el 50.0 por ciento presentó afectación de mucosa oral. De todos los pacientes el 33.3 por ciento presentaron remisión de su enfermedad siendo las complicaciones más frecuentes las piodermitis (37.5 por ciento) neumonía (17.2 por ciento) ynecrosis aséptica de cadera (17.2 por ciento). CONCLUSIONES: El PF constituye el tipo de pénfigo más frecuente. El pénfigo en el HNDM predomina en varones y adultos de edad media; son frecuentes las formas endémicas tanto para el PF como parael PV.

Aim: To determine clinical, epidemiological and laboratory characteristics of patients with pemphigus at Dos de Mayo National Hospital (HNDM) during 1998- 2007. Material and methods: A descriptive and retrospective study. Population was constituted by patients with diagnosis of pemphigus foliaceus (PF), pemphigus vulgaris (PV) and paraneoplastic pemphigus (PNP) at the HNDM between1998-2007. Clinical histories of patients were revised, obtaining clinical, epidemiological and laboratory dates, which were registered in an instrument of recollection of dates. Results: There were 38 cases of pemphigus, 29 corresponded PF, 8 PV and 1 PP. 22 of 29 patients with PF (75.9 per cent) corresponded non endemic form and the rest (24.1 per cent) to the endemic form. Concerning evolution, 27.3 per cent of patients presented remissionof their diseases and the more frequent complications were pyoderma (24.1 per cent), pneumonia (17.2 per cent) and aseptic necrosis of the femur head (17.2 per cent). 2 (25.0 per cent) of 8 patients diagnosed with PV corresponded to endemic form, 50.0 per cent presented affectation of oral mucosa. 33.3 per cent of patients presented remission of their diseases, being the most frequent complication pyoderma (37.5 per cent). Conclusions: PF constitutes the most frequent type of pemphigus. Pemphigus at HNDM is greater in male and middle age adults; endemic forms are frequent for PF and PV.

Pénfigo foliáceo endémico en el Perú: caracterización clínica, epidemiológica e inmunopatológica / Endemic pemphigus foliaceus in Perú: clinical, epidemiological and immunopathologic characterization

Objetivo: Determinar las características clínicas, epidemiológicas e inmunopatológicas de los pacientes con pénfigo foliáceo endémico, en el Perú. Pacientes y métodos: Estudio comparativo, prospectivo y multicéntrico realizado de julio de 2002 a junio de 2006. La muestra estuvo constituida por 52 pacientes con pénfigo foliáceo endémico, captados en el Hospital Regional de Pucallpa, Hospital de Apoyo de Yarinacocha, Puesto de Salud de Pueblo Libre (Ucayali), Hospital Nacional Dos de Mayo (Lima), y mediante búsqueda activa. Se registró las características clínicas y epidemiológicas; el estudio inmunopatológico (Elisa, inmunoprecipitación e inmunofluorescencia indirecta) se realizó en 16 pacientes con pénfigo foliáceo endémico, 41 sujetos sanos de un área endémica (Pueblo Libre), 11 familiares de pacientes y 20 donantes sanos procedentes de áreas no endémicas de Pucallpa, Lima y Chachapoyas. Resultados: El pénfigo foliáceo endémico es endémico en Ucayali, Loreto, Huánuco, Amazonas, Junín y San Martín; la edad promedio de los pacientes fue 31,4 ± 18,3 años y 57,7 por ciento correspondió al sexo masculino. El 65,4 por ciento presentó la forma clínica generalizada y el resto la forma localizada. Se detectó anticuerpos antidesmogleína 1 mediante Elisa en 13/16 pacientes (81,3 por ciento) y antidesmogleína 3, en 5/16 (31,3 por ciento). Los pacientes con pénfigo foliáceo endémico presentaron anticuerpos antidesmogleína 1 de tipo IgG4, IgG1 e IgG2. Los sujetos sanos del foco endémico de Pueblo Libre y los familiares de los pacientes con pénfigo foliáceo endémico también desarrollaron anticuerpos antidesmogleína 1 y 3. Conclusiones: El pénfigo foliáceo endémico, en el Perú, es una enfermedad que se presenta en adolescentes y adultos jóvenes, siendo la forma clínica predominante la generalizada...

Objective: To determine clinical, epidemiological and immunopathologic characteristics of endemic pemphigus foliaceus in Peru. Patients and methods: Prospective and multicentric study from July 2002 through June 2006. The sample consisted in 52 patients with endemic pemphigus foliaceus seen in the following centers: Pucallpa Regional Hospital, Yarinacocha Hospital, Pueblo Libre Health Center (Ucayali), Hospital Nacional Dos de Mayo (Lima) and by active research. Clinical and epidemiological characteristics were registered; immunopathologic study (Elisa, immunoprecipitation, and indirect immunoflourescence tests) was performed in 16 patients with endemic pemphigus foliaceus, in 41 healthy individuals of an endemic area (Pueblo Libre community), 11 patients’ relatives and in 20 healthy donors from non endemic areas of Pucallpa, Lima and Chachapoyas.Results: endemic pemphigus foliaceus is endemic in Ucayali, Loreto. Huanuco, Amazonas, Junin and San Martin. Patients’ mean age was 31,4 ± 18,3 years and 57,7% were male. The generalized presentation occurred in 65,4% of patients. Anti-desmoglein 1 antibodies were detected by Elisa in 13 of 16 patients (81,3%) and anti-desmoglein 3 in 5 of 16 (31,3%). Patients with endemic pemphigus foliaceus presented IgG4, IgG1 and IgG2 antidesmoglein antibodies. Moreover healthy subjects of the endemic focus of the Pueblo Libre community and the patients’ relatives developed antibodies against desmoglein 1 and 3.Conclusions: In Peru, endemic pemphigus foliaceus is a disease that occurs in adolescents and young adults; the predominant clinical presentation is generalized. The immunologic profile of patients with endemic pemphigus foliaceus is predominantly IgG4. Healthy subjects of endemic areas and patients’ relatives developed anti-desmoglein 1 and 3 antibodies.

Pênfigo foliáceo endêmico: características sociodemográficas e incidência nas microrregiões do estado de Goiás, baseadas em estudo de pacientes atendidos no Hospital de Doenças Tropicais, Goiânia, GO / Endemic pemphigus foliaceus: social and demographical characteristics and incidence in the microregions of Goias, based on patients seen at the Tropical Diseases Hospital, Goiania, Goias

Fundamento: o pênfigo foliáceo endêmico é doença auto-imune, cutânea, bolhosa, com incidência maior na região Centro-Oeste do Brasil e menor em alguns países sul-americanos. Embora tenha sido demonstrado seu caráter auto-imune pela presença de auto-anticorpos e a importância da predisposição genética, não estão ainda claramente estabelecidos os fatores ambientais intervenientes

Situação do pênfigo foliáceo endêmico em Mato Grosso do Sul, Brasil, 1990-1999 / Situation of endemic pemphigus foliaceus in Mato Grosso do Sul, Brazil, 1990-1999

Com o objetivo de caracterizar epidemiologicamente o pênfigo foliáceo endêmico, investigaram-se 307 casos. A patologia predominou no trabalhador rural na faixa de 10 a 42 anos. Verificou-se maior incidência em dezembro, janeiro, fevereiro, março e maio (média de 30,7 casos anuais). Os achados evidenciaram um corredor coincidindo com o divisor de águas do estado