Pénfigo foliáceo, variedad seborreica, reporte de caso / Pemphigus foliaceus, seborrheic variety, case report

Presentación del caso. Se expone el caso de un paciente masculino de 48 años de edad, sin antecedentes médicos conocidos, que presentó múltiples lesiones en forma de placas eritematocostrosas fácilmente descamativas, inicialmente en tórax anterior, que se esparcían sobre el rostro y cuero cabelludo sin afectar las mucosas. Intervención terapéutica. El manejo hospitalario se basó fundamentalmente en el uso de esteroides tópicos y sistémicos, así como el manejo de las infecciones sobreagregadas a las lesiones dermatológicas y el apoyo psicológico del paciente. Se tomó biopsia de piel donde se evidenció la presencia de acantólisis, confirmando el diagnóstico de esta enfermedad autoinmunitaria. Evolución clínica. Luego del tratamiento se logró una reducción de las múltiples lesiones descamativas, el control de la infección local y la recuperación de la piel del paciente, la cual a pesar de aún presentar cicatrices se encontraba con sus funciones restituidas

Case presentation. a 48-year-old male with no known medical history who presented multiple lesions in the form of easily desquamative erythematous and crusted plaques, initially on the anterior thorax, which spread over the face and scalp without affecting the mucous membranes. Treatment. In-hospital management was mainly based on topical and systemic steroids, the management of infections superadded to the dermatologic lesions, and psychological support for the patient. A skin biopsy was taken where acantholysis was evidenced, confirming the autoimmune disease diagnosis. Outcome.After treatment, the multiple scaly lesions were reduced, the local infection was controlled, and the patient's skin recovered although it still had scars, its functions were restored

Oral lesions after infection by SARS CoV 2: two case reports / Lesiones bucales posteriores a infección por SARS CoV 2 presentación de dos casos

Introduction: The SARS CoV 2 infection has resulted in several health, economic, and social crises in all areas. The disease shows a substantial biological diversity in humans causing a series of sequels in the trans- or post-infection period in the entire organism. Case Report: The manifestations that occur in the oral cavity and pharynx have not been evaluated. In this study, two clinical cases are reported. The first patient, a 67-year-old male, presents erosive lesions on the dorsal surface of his tongue after SARS CoV 2 infection. Results: Therapy consisting of reinforcing oral cleaning, use of antifungal solutions, mouthwashes containing superoxidation solution and B complex was given to the patient. The reported lesions improved satisfactorily. The second case, a 47-year-old male patient, presented vesiculobullous lesions on the lingual and labial mucosa accompanied by severe painful symptoms after SARS CoV 2 infection. An incisional biopsy was performed. The histopathological result was compatible with pemphigus vulgaris, and the treatment protocol was started with 0.1% topical mometasone and 2g miconazole gel, observing adequate involution of the lesions after 20 days. Conclusions: The aim of this study is to report on the lesions affecting the oral cavity and pharynx in post-COVID patients with the aim of carrying out a thorough intraoral examination, establishing a clinical or histopathological diagnosis to implement a specific treatment plan in each case to improve the health and quality of life of the patients. Keywords: SARS-CoV-2; Oral manifestations; Oral ulcer; Pemphigus; Mouth; Mucous membrane.

Introducción: La infección por virus de SARS CoV 2 ha dejado a su paso una estela de crisis en materia de salud, económica, social y en todos los ámbitos a la fecha seguimos realizando la observación del comportamiento de la enfermedad en los seres humanos con una diversidad biológica importante y que ha traído como consecuencia una serie de secuelas que se presentan en el periodo trans o posterior a la infección en toda la economía corporal. Reporte de Caso: Se ha evaluado poco las manifestaciones que se presentan en la cavidad bucal y faringe; se presentan dos casos clínicos el primero paciente masculino de 67 años de edad posterior a la infección por SARS CoV 2 presenta diluciones de continuidad en bordes laterales de la lengua se indica terapia y refuerza limpieza bucal, antimicótico, colutorios con solución de superoxidación y complejo B, las úlceras involucionan de manera satisfactoria. Resultados: El segundo caso masculino de 47 años posterior a la infección por SARS CoV 2 debuta con lesiones vesículo-ampollosas en mucosa lingual, labial con sintomatología dolorosa severa, se realiza biopsia incisional donde el resultado histopatológico es compatible con pénfigo vulgar, se inicia protocolo de tratamiento con mometasona tópica al 0.1% y miconazol gel 2g observándose una adecuada involución de las lesiones a los 20 días. Conclusiones: El objetivo de este trabajo es poner en contexto de la comunidad médica y científica las lesiones concernientes a la cavidad bucal y faringe que están presentando los pacientes postcovid con el objetivo de realizar una exhaustiva exploración intraoral, establecer un diagnóstico clínico o histopatológico y con base en esto instaurar un plan de tratamiento específico en cada caso en particular con el fin fundamental de mejorar la salud y calidad de vida del paciente.

Penfigoide gestacional. Reporte de caso

El penfigoide gestacional, antes conocido como herpes gestacional, es una dermatosis rara, ampollosa, autoinmune y específica del embarazo. Aunque la etiología no se conoce del todo, la mayoría de los pacientes desarrollan anticuerpos contra una proteína hemidesmosomal transmembrana de 180 kDa. Los síntomas que definen la enfermedad son el picor intenso y las lesiones urticariales que evolucionan hacia ampollas dolorosas. Las lesiones comienzan en la región periumbilical en el 90% de los casos y se extienden rápidamente a otras zonas del cuerpo, a excepción de la cabeza y las mucosas. Es más frecuente en el segundo o tercer trimestre. En las muestras de biopsia de piel, la tinción de inmunofluorescencia directa valida el diagnóstico. Los corticoides tópicos pueden ser utilizados para tratar los síntomas leves, mientras que los corticoides orales y los antihistamínicos deben usarse para tratar los casos graves. Se presenta un caso de penfigoide gestacional.

Pemphigoid gestationis, formerly known as herpes gestationis, is a rare, blistering, autoimmune, pregnancy-specific dermatosis. Although the etiology is not fully understood, most patients develop antibodies against 180 kDa transmembrane hemidesmosomal protein. The defining symptoms of the disease are intense itching and urticarial lesions that evolve into painful blisters. Lesions begin in the periumbilical region in 90% of cases and spread rapidly to other areas of the body, except for the head and mucous membranes. It is most frequent in the second or third trimester. In skin biopsy specimens, direct immunofluorescence staining validates the diagnosis. Topical corticosteroids can be used to treat mild symptoms, while oral corticosteroids and antihistamines should be used to treat severe cases. A case of pemphigoid gestationis is presented.

Pénfigo vulgar y su manejo integral: reporte de dos casos y revisión de literatura / Pemphigus Vulgaris and its Comprehensive Management: Report of Two Cases and Literature Review

Resumen En la cavidad oral se pueden presentar lesiones en gíngiva que no están asociadas a placa bacteriana, las cuales requieren de un adecuado diagnóstico y tratamiento. La gingivitis descamativa está usualmente relacionada con desórdenes mucocutáneos, como el pénfigo vulgar (PV), donde las lesiones orales incluyendo las lesiones gingivales, pueden preceder las lesiones cutáneas. El manejo es multidisciplinario y el tratamiento incluye terapia farmacológica tópica y sistémica, se requiere un adecuado control de la placa bacteriana por parte del paciente y una estricta supervisión en el tiempo por parte del profesional para el mantenimiento y estabilidad de los tejidos gingivales. Se presentan dos casos clínicos de pacientes con diagnóstico de PV los cuales fueron manejados de manera oportuna e integral para controlar y estabilizar el factor sistémico y local.

Abstract In the oral cavity, gingiva lesions may occur that are not associated with bacterial plaque, which require adequate diagnosis and treatment. Desquamative gingivitis is usually related to mucocutaneous disorders, such as pemphigus vulgaris (PV), where oral lesions, including gingival lesions, may precede skin lesions. Management is multidisciplinary and treatment includes topical and systemic pharmacological therapy, require adequate control of dental plaque by the patient and strict supervision over time by the professional for the maintenance and stability of the gingival tissues. Two clinical cases of patients with a diagnosis of PV are presented, which were managed in a timely and integral way to control and stabilize the systemic and local factor.

Pênfigo vegetante: abordagem cirúrgica como tratamento complementar / Pemphigus vegetans: surgical approach as a complementary treatment

O pênfigo vegetante é considerado uma rara variante clínica do pênfigo vulgar e cursa com lesões recorrentes que podem evoluir para placas vegetantes extensas de difícil resolução. A cirurgia pode ser uma alternativa no tratamento complementar de lesões resistentes à terapêutica clássica. Há escassez de literatura que evidencie a técnica cirúrgica e os resultados pós-operatórios da abordagem do pênfigo vegetante, tornando relevante a comunicação deste caso. Apresentamos o caso de um paciente com pênfigo vegetante de Hallopeau, tratado com sucesso por meio da exérese tangencial seguida da cicatrização por segunda intenção de grandes projeções retroauriculares

Pemphigus vegetans is considered a rare clinical variant of pemphigus vulgaris and is associated with recurrent lesions that can evolve into extensive vegetative plaques that are difficult to resolve. Surgery may be an alternative in the complementary treatment of lesions resistant to classical therapy. There is a shortage of literature that shows the surgical technique and the postoperative results of the approach to pemphigus vegetans, making the communication of this case relevant. We present the case of a patient with the Hallopeau type of pemphigus vegetans successfully treated with tangential exeresis followed by secondary intention healing of large retroauricular projections.

Diagnósticos de enfermagem no pênfigo: revisão integrativa da literatura / Nursing diagnoses in pemphigus: integrative literature review

Pênfigos são um grupo de doenças bolhosas, autoimunes, de etiologia desconhecida, que acometem a pele e as mucosas. Objetivo: levantar Diagnósticos de Enfermagem para pacientes com Pênfigo, após Revisão Integrativa da Literatura, segundo a NANDA-I, versão 2021/2023. Método: Revisão Integrativa da Literatura, realizada em junho de 2021, de artigos publicados de 2011 à 2021, indexados nas bases de dados: Biblioteca Virtual em Saúde, Literatura Latino-Americana e do Caribe em Ciências da Saúde, Scientific Electronic Library Online, US National Library of Medicine, Medical Literature Analysis and Retrieval System Online, Biblioteca Virtual Em Salud Enfermaría. A amostra inicial foi composta por 65 artigos e a final por 5 (cinco) artigos, sendo excluídos os que não atendiam aos critérios de inclusão e que não respondiam as perguntas norteadoras da pesquisa. Resultado: houve predomínio das publicações nacionais, em 2016, realizadas por enfermeiros. Nos artigos da amostra, foram encontrados 24 Diagnósticos de Enfermagem. Desses, 1 (um) foi excluído e 9 (nove) atualizados de acordo com Referencial Teórico. Foram também acrescentados 9 (nove) Diagnósticos de Enfermagem conforme os sinais e sintomas encontrados nos artigos da Revisão. Conclusão: nota-se que o Referencial Teórico, embora recentemente atualizado, ainda requer a abordagem de temas referentes às patologias dermatológicas. Evidenciou-se ainda a necessidade de realizar novos estudos sobre os Diagnósticos e cuidados de Enfermagem utilizados na assistência prestada aos pacientes com Pênfigo, devido a escassez de materiais publicados.

Conhecimento da enfermagem sobre os cuidados com paciente portador de pênfigo / Nursing knowledge about the care of patients with pemphigus

A palavra pênfigo envolve um grupo de doenças bolhosas autoimunes que atingem a pele e as mucosas. Existem três tipos predominantes: o pênfigo vulgar, o pênfigo foliáceo e o penfigóide bolhoso. O conhecimento sobre a doença e seus cuidados é essencial para o atendimento a esses pacientes, e para isso é necessário a elaboração de um plano de cuidados diários e contínuos. Objetivo: descrever o conhecimento da equipe de enfermagem sobre os cuidados com o paciente portador de pênfigo, comparar o conhecimento da equipe de enfermagem com o descrito na literatura e construir um protocolo de cuidados de enfermagem para esses pacientes.Método: estudo quantitativo, descritivo, que buscou respostas da equipe de enfermagem sobre o conhecimento e cuidados com o paciente portador de pênfigo por meio de um questionário aplicado em 2021 no "Instituto Lauro de Souza Lima".Resultados: A maioria dos profissionais eram auxiliares de enfermagem por um longo tempo e com experiência na área de dermatologia. Demonstraram conhecimento teórico e prático em relação à doença e dúvidas em relação a alguns aspectos, como: tipo de isolamento (reverso x contato), higiene oral, cobertura, tempo de troca de cateter venoso nos pacientes portadores de pênfigo. Conclusão: Os aspectos levantados pela pesquisa precisam ser abordados, treinados e acompanhados pelos enfermeiros nas instituições. O protocolo de cuidados de enfermagem para pacientes portadores de pênfigo poderá ser utilizado para treinamento da equipe de enfermagem

The word pemphigus involves a group of bullous autoimune diseases which affect the skin and mucous membranes. There are three predominant types of pemphigus: pemphigus vulgaris, pemphigus foliaceus and bullous pemphigoid. Knowledge about the disease and care becomes essential for the treatment of these patients, and it iss necessary to develop a daily and continuous care plan. Objective: To describe the knowledge of the nursing team about the care of patients with pemphigus, to compare the knowledge of the nursing team with the description in the literature, as well as to present a nursing care protocol which may improve the quality of life of patients affected by pemphigus through the elaboration of a nursing care protocol for these patients. Method: a quantitative, descriptive study which sought answers from the nursing team about the knowledge and care of the patient with pemphigus, through a questionnaire applied in 2021 at "Lauro de Souza Lima Institute". Results: Most professionals were nursing assistants, with years of work and experience in the area of dermatology, demonstrated theoretical and practical background about the disease, and issues regarding the following aspects, such as: type of isolation (reverse x contact), oral hygiene, dressing how long should a venous catheter be changed, regarding patients with pemphigus. Conclusion: The aspects raised by the research need to be addressed, trained and monitored by nurses in the Institutions. The nursing care protocol for patients with pemphigus can be employed to train the nursing team.

Estudo clínico e epidemiológico dos casos de pênfigo foliáceo endêmico e pênfigo vulgar em um centro de referência no estado de Minas Gerais, Brasil / Clinical and epidemiological study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil

Fundamentos: o pênfigo é um grupo de doenças bolhosas autoimunes, sendo as mais comuns o pênfigo vulgar e o pênfigo foliáceo, esse último predominando no Brasil na forma endêmica e conhecido como fogo selvagem. São caracterizados pela produção de anticorpos IgG contra desmogleínas. É descrita redução na incidência do pênfigo foliáceo endêmico e aumento do pênfigo vulgar no país, mas não há estudos em Minas Gerais que abordem este último. Objetivo: descrever o perfil epidemiológico e clínico dos pacientes com pênfigo foliáceo e vulgar atendidos no serviço de dermatologia de um hospital universitário público de Minas Gerais, Brasil. Métodos: realizou-se estudo observacional, descritivo e transversal dos casos com diagnóstico de pênfigo foliáceo endêmico ou vulgar, pelo período de seis meses. Foi preenchido um questionário com dados epidemiológicos e clínicos da doença. Resultados: foram incluídos 122 pacientes no estudo, 64 com pênfigo foliáceo e 58 com pênfigo vulgar. Ao se comparar os pacientes com pênfigo foliáceo endêmico e vulgar, a mediana da idade de manifestação inicial da doença foi menor no foliáceo (p=0,001); as profissões dos pacientes com pênfigo foliáceo predominam no setor primário e, com pênfigo vulgar, no setor terciário (p=0,010); a maioria do grupo com pênfigo foliáceo residia na zona rural e periurbana, enquanto com pênfigo vulgar, na zona urbana (p=0,000); vegetação (p=0,000) e rios/córregos próximos à moradia (p=0,001) são relatados com maior frequência pelos pacientes com pênfigo foliáceo; e a mediana da quantidade de medicações sistêmicas necessárias para controle da doença é maior no pênfigo vulgar (p=0,002). Ao se comparar os pacientes com pênfigo foliáceo endêmico àqueles avaliados em estudo realizado no serviço em 2008, no estudo atual a maioria é de zona periurbana e rural, enquanto no anterior, de área urbana (p=0,030). Limitações do estudo: a população avaliada advém de um serviço de atenção terciária que não é referência para todo estado. Conclusões: os pacientes com pênfigo foliáceo endêmico e vulgar mantêm diferenças estatisticamente significativas nas principais variáveis já descritas na literatura, como idade e zona de moradia. Historicamente, é percebida uma redução de casos de pênfigo foliáceo endêmico e aumento de casos de pênfigo vulgar nos pacientes dessa instituição.

Background: pemphigus is a group of bullous autoimmune diseases. The most common of which are pemphigus vulgaris and pemphigus foliaceus, the latter predominating in Brazil in the endemic form, also known as "fogo selvagem". They are characterized by the production of IgG antibodies against desmogleins. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris have been described in the country, but there are no studies in Minas Gerais that address the latter. Objective: to describe the epidemiological and clinical profile of patients with pemphigus foliaceus and vulgaris treated at the dermatology service of a public university hospital in Minas Gerais, Brazil. Methods: an observational, descriptive and cross-sectional study of cases diagnosed with vulgar or endemic pemphigus foliaceus was carried out for a period of six months. A questionnaire with epidemiological and clinical data on the disease was filled out. Results: a total of 122 patients were included in this study, 64 with pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and pemphigus vulgaris, the median age at onset of the disease was lower in the foliaceus (p=0.001); the professions of patients with pemphigus foliaceus predominate in the primary sector and, with pemphigus vulgaris, in the tertiary sector (p=0.010); most of the pemphigus foliaceus group lived in rural and periurban areas, while those with pemphigus vulgaris lived in urban areas (p=0.000); vegetation (p=0.000) and rivers/streams close to the home (p=0.001) are more frequently reported by patients with pemphigus foliaceus; and the median amount of systemic medications needed to control the disease is higher in pemphigus vulgaris (p=0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the service in 2008, in the current study most are from periurban and rural areas, while in the previous one, from urban areas (p=0.030). Study limitations: the evaluated population comes from a tertiary care service that is not a reference for the entire state. Conclusions: patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences in the main variables described in the literature, such as age and housing area. Historically, a reduction in endemic pemphigus foliaceus cases and an increase in cases of pemphigus vulgaris among the patients of this institution have been observed.

Sífilis congénita atípica con doble VDRL negativo materno. Reporte de un caso / Atypical congenital syphilis with a double negative VDRL from the mother. A case report

Resumen INTRODUCCIÓN: La sífilis es una infección de trasmisión sexual adquirida a través de una trasfusión sanguínea, por contacto directo con una lesión activa o por vía vertical. La infección puede trasmitirse al feto en cualquier momento del embarazo. Cada año se registran en el mundo 749,000 casos de sífilis congénita. En México, del 2007 al 2017, se reportaron 1030 casos de sífilis congénita (media de 90.6 casos por año). En 50 a 80% de los casos hay complicaciones materno-fetales. OBJETIVO: Reportar un caso clínico de sífilis congénita temprana, con pénfigo bulloso y descamación al nacimiento. CASO CLÍNICO: Paciente de 25 años, con antecedente de cuatro embarazos, dos partos y un aborto, previamente sana, con control prenatal y pruebas de VDRL y VIH negativas. Los dos últimos embarazos finalizaron con recién nacido, ambos de sexo femenino, con lesiones ampollosas decapitadas, placas blanquecinas de aspecto áspero elevadas sobre una base eritematosa, eritema con descamación gruesa en las manos y pies, y descamación fina generalizada. Ante la sospecha de sífilis congénita se inició la administración de 50,000 UI/kg de penicilina G cristalina por vía intravenosa cada 12 h. La sospecha de sífilis congénita se confirmó. La paciente se dio de alta con citas de seguimiento en la consulta externa. CONCLUSIONES: El control prenatal adecuado, con una o dos pruebas para sífilis, no es suficiente para prevenir la forma congénita. Es necesario el análisis de los casos para encontrar opciones e implementar estrategias de salud pública que prevengan nuevos casos.

Abstract INTRODUCTION: Syphilis is a sexually transmitted infection acquired through blood transfusion, by direct contact with an active lesion or by the vertical route. The infection can be transmitted to the fetus at any time during pregnancy. Each year, 749,000 cases of congenital syphilis are reported worldwide. In Mexico, from 2007 to 2017, 1030 cases of congenital syphilis were reported (average of 90.6 cases per year). In 50 to 80% of cases there are maternal-fetal complications. OBJECTIVE: To report a clinical case of early congenital syphilis with bullous pemphigus and desquamation at birth. CLINICAL CASE: 25-year-old patient, with a history of four pregnancies, two deliveries and one abortion, previously healthy, with prenatal control and negative VDRL and HIV tests. The last two pregnancies ended with a newborn, both female, with decapitated blistering lesions, whitish plaques of rough appearance raised on an erythematous base, erythema with thick desquamation on the hands and feet, and generalized fine desquamation. Suspecting congenital syphilis, 50,000 IU/kg of crystalline penicillin G intravenously every 12 h was started. The suspicion of congenital syphilis was confirmed. The patient was discharged with follow-up outpatient appointments. CONCLUSIONS: Adequate prenatal screening, with one or two tests for syphilis, is not sufficient to prevent the congenital form. Case analysis is necessary to find options and implement public health strategies to prevent new cases.

Recién nacido con pénfigo sifilítico en tiempos de pandemia / Newborn with syphilitic pemphigus in pandemic's time

Resumen La sífilis congénita es la infección del recién nacido (RN) por Treponema palllidum transmitida durante el embarazo desde la madre al feto. Actualmente, sigue siendo una causa importante de morbi-mortalidad fetal prevenible en todo el mundo, pese a que con un tratamiento adecuado y oportuno en la madre, se logra revertir en forma efectiva los resultados adversos en el feto y RN. Lo anterior destaca la importancia del control del embarazo, y la pesquisa precoz de la infección materna. Si bien, Chile ha experimentado un aumento de la incidencia de sífilis en la población general, se ha mantenido una tasa estable de sífilis congénita de 0,1 por 1.000 RN vivos. Presentamos el caso clínico de un RN, hijo de una madre sin control del embarazo, que presentó lesiones compatibles con un pénfigo sifilítico.

Abstract Congenital syphilis is the infection by Treponema pallidum of the newborn, due to mother-to-child transmission of spirochaetes during pregnancy. It remains as a major cause of preventable fetal and neonatal morbidity and mortality if the mother is not opportunely treated. This highlights the importance of an adequate prenatal control, early screening for maternal infection and timely treatment. Although Chile has experienced an increase in the incidence of syphilis in the general population, a stable rate of congenital syphilis in 0.1/1.000 live births has been maintained. In this case report, we present a newborn whose mother did not have antenatal control and showed dermatological lesions compatible with syphilitic pemphigus.

Pénfigo vulgar mucoso: a propósito de un caso. Revisión de la literatura / Mucous pemphigus vulgaris: a case report

Introducción: El Pénfigo Vulgar (PV) es una enfermedad de origen autoinmune caracterizada por causar ampollas intraepidérmicas en piel y mucosas, como consecuencia de la agresión de autoanticuerpos hacia varios tipos de proteínas desmosómicas. El elemento eruptivo primordial es la ampolla, que puede presentarse de manera individual o en coalescencia con la consecuente formación de placas erosivo-costrosas. En el 90% de los casos las lesiones afectan a la mucosa oral, mientras que en el 50-70% de los mismos constituyen la primera manifestación de la enfermedad. Objetivo: Se presenta un caso clínico de PV y una revisión bibliográfica actualizada, con el objetivo de analizar sus factores etiológicos y sus opciones terapéuticas. Caso clínico: Se presenta el caso de un paciente varón, de 71 años, fumador, con condición prediabética y síndrome de Guillain Barré remitido al Servicio de Cirugía Bucal e Implantología del Hospital Virgen de la Paloma de Madrid con un cuadro clínico caracterizado por infección oral y pérdida de peso. Una vez confirmado el diagnóstico de PV mucoso mediante examen histopatológico, se estableció una terapia con corticoides sistémicos obteniendo una remisión casi completa de las lesiones. Tras varias semanas de tratamiento su dermatólogo decidió suspender los corticoides para llevar a cabo, sin éxito, una terapia sustitutiva con inmunosupresores. La recidiva de las lesiones, unida a los efectos adversos causados por la nueva terapia, obligó a reconsiderar la suministración de corticoides con una resolución positiva de la enfermedad. (AU)

Introduction: Pemphigus Vulgaris is an autoimmune disease characterized by causing intraepidermal blisters on the skin and mucosa, as a consequence of the aggression of autoantibodies towards various types of desmosomal proteins. The primary eruptive element is the blister, which can appear in coalescence with the consequent formation of erosive-crusted plaques. In 90% of cases lesions affect the oral mucosa, while in 50-70% they are the first manifestation of the disease. Objective: We aim to report a case of Pemphigus Vulgaris and an updated literature review to analyse its etiological factors and treatment options. Clinical case: We present the case of a 71-year-old male patient, smoker, with prediabetic condition and sindrome Guillain Barré referred to the Oral Surgery and Implantology Service of the Virgen de la Paloma Hospital in Madrid with a clinical picture characterized by oral infection. Once the diagnosis of mucosal PV was confirmed, a systemic corticosteroid therapy was established, obtaining almost complete remission of the lesions. After several weeks of treatment, his dermatologist decided to suspend the corticosteroids to carry out unsuccessful immunosuppressant replacement therapy. The recurrence of the lesions, together with the adverse effects caused by the new therapy, forced the reconsideration of the supply of corticosteroids with a positive resolution of the disease. (AU)

Paraneoplastic pemphigus as an atypical presentation of Castleman disease / Pénfigo paraneoplásico como presentación atípica de la enfermedad de Castleman

Abstract Castleman disease is a non-clonal lymphoproliferative disorder with a broad range of clinical manifestations. We present the case of a male patient with a clinical picture of asthenia, adynamia, hyporexia, weight loss, oral and genital ulcers, and red, itchy eyes. The physical exam showed conjunctival redness and oral and genital ulcers. Computed axial tomography with contrast of the chest and abdomen revealed multiple enlarged mediastinal and retroperitoneal lymph nodes, and a solid 94x51 mm retroperitoneal mass. A biopsy of the mass was taken, which reported the hyaline vascular variant of Castleman disease. A scrotal lesion biopsy was also ordered, with a histopathological analysis compatible with pemphigus. In addition, direct immunofluorescence was positive in the epidermal intercelullar spaces, as well as immunoprecipitation with anti-desmoglein, anti-desmoplakin, anti-envoplakin and pemphigoid ampule antigen. Thus, the presence of multicentric Castleman disease associated with paraneoplastic pemphigus was established. (Acta Med Colomb 2021; 46. DOI: https://doi.org/10.36104/amc.2021.1964)

Resumen La enfermedad de Castleman es un trastorno linfoproliferativo no clonal con amplia gama de manifestaciones clínicas. Se presenta el caso de paciente masculino con cuadro clínico consistente en astenia, adinamia, hiporexia, pérdida ponderal, úlceras orales y genitales, prurito ocular e hi peremia conjuntival. El examen físico evidenció hiperemia conjuntival, úlceras orales y genitales. La tomografía axial computarizada contrastada de tórax y abdomen reveló múltiples adenopatías mediastinales, retroperitoneales y masa sólida de 94 x 51 milímetros de localización retroperitoneal. Se realizó biopsia de la masa previamente descrita, que reportó enfermedad de Castleman variante hialino vascular. También se indicó biopsia de lesión escrotal cuyo análisis histopatológico fue compatible con pénfigo, además la fluorescencia inmunológica directa fue positiva en los espacios intercelulares de la epidermis al igual que inmunoprecipitación con anticuerpos anti-desmogleina, anti-desmoplaquina, anti-envoplaquina y antígeno del penfigoide ampollar. Por lo anteriormente descrito se definió la existencia de enfermedad de Castleman multicéntrica asociada a pénfigo paraneoplásico. (Acta Med Colomb 2021; 46. DOI: https://doi.org/10.36104/amc.2021.1964)

Pênfigo Vulgar Oral em Idosa: Condutas de um Caso Desafiador / Oral Pemphigus Vulgaris in Old Woman: Conducts of a Challenging Case

A doença pênfigo é uma enfermidade autoimune que dependendo do nível de acometimento epitelial, pode ser classificada em pênfigo vulgar, vegetante, eritematoso e foliáceo. Além desses, há também o denominado pênfigo paraneoplásico, que ocorre especialmente em casos que há vínculo a neoplasias malignas com destaque para os linfomas. Somente os tipos vulgar e vegetante podem afetar a mucosa oral, sendo o pênfigo vulgar o mais prevalente. O mecanismo fisiopatológico da doença é caracterizado pela ação de autoanticorpos contra as proteínas desmogleínas dos desmossomos encontrados nas células epiteliais, desencadeando assim, a formação de fendas intraepiteliais e bolhas. O manejo dos pacientes com pênfigo vulgar oral é bastante desafiador, especialmente em casos de indivíduos idosos e portadores de comorbidades. O presente trabalho objetiva discutir aspectos contemporâneos do pênfigo vulgar oral e elucidar o caso de uma idosa acometida pela doença, destacando toda a propedêutica utilizada no seu atendimento e o tratamento empregado, com o uso de corticoides sistêmicos e acompanhamento constante da condição da paciente, uma vez que o pênfigo vulgar é uma doença que não tem cura.

Pemphigus is an autoimmune disease that, depending on the level of epithelial involvement, can be classified into pemphigus vulgaris, vegetans, erythematosus and foliaceus. In addition to these, there is also the so-called paraneoplastic pemphigus, which occurs especially when there is a link to malignant neoplasms with emphasis on lymphomas. Only the vulgaris and vegetans types can affect the oral mucosa, with pemphigus vulgaris being the most prevalent one. The pathophysiological mechanism of the disease is characterized by the action of autoantibodies against the desmoglein proteins of the desmosomes found in the epithelial cells, thus triggering the formation of intraepithelial clefts and blisters. The management of patients with oral pemphigus vulgaris is quite challenging, especially in cases of older individuals and patients with comorbidities. This study discusses contemporary aspects of oral pemphigus vulgaris and elucidate the case of an old woman affected by the disease, highlighting all the propaedeutics used in her care and the treatment employed, with the use of systemic corticosteroids and constant follow-up of the patient's condition, since pemphigus vulgaris is a disease that has no cure.

Pénfigo benigno familiar de presentación atípica en paciente sin antecedente familiar / Atypical presentation of familial benign pemphigus in patient without familiar background

Resumen La enfermedad de Hailey-Hailey, también llamada pénfigo familiar benigno, corresponde a una genodermatosis debilitante que se transmite mediante un patrón autosómico dominante, con una prevalencia de alrededor de 1 en 50.000 casos. El reporte de antecedentes familiares está presente hasta en 60 % de los pacientes. Se caracteriza por la presencia de vesículas crónicas y recurrentes, erosiones y exulceraciones en zonas de flexura. El tratamiento puede representar un reto, porque a pesar del manejo con terapias tópicas, corticosteroides sistémicos, inmunomoduladores sistémicos y el empleo de láser, ninguna terapia ha logrado una remisión a largo plazo. Se presenta el caso de un paciente masculino, adulto medio, sin antecedente familiar alguno, con historia de placas de superficie descamativa y hematocostras recurrentes crónicas y presentación clínica atípica, dada la localización de lesiones predominantes en miembros superiores, con sospecha inicial de psoriasis vulgar, con posterior toma de biopsia y reporte de patología que evidencia histológia típica de PBF. Por lo cual se indica manejo con corticosteroides sistémicos, sin evidencia de reacciones adversas y con remisión a largo plazo. MÉD.UIS.2020;34(1):101-6

Abstract Hailey-Hailey disease, also called benign familial pemphigus, corresponds to a debilitating genodermatosis that is transmitted through an autosomal dominant pattern, with a prevalence of around 1 in 50,000 cases. The family history report is present in up to 60% of patients. It is characterized by the presence of chronic and recurrent vesicles, erosions and exulcerations in flexural areas. Treatment can be challenging, because despite management with topical therapies, systemic corticosteroids, systemic immunomodulators, and the use of lasers, no therapy has achieved long-term remission.We present the case of a male patient, middle adult, without any family history, with a history of scaly surface plaques and chronic recurrent hematocostras and atypical clinical presentation given the location of predominant lesions in the upper limbs, with initial suspicion of vulgar psoriasis, with subsequent biopsy and pathology report showing typical PBF histology. Therefore, management with systemic corticosteroids without evidence of adverse reactions and with long-term remission is indicated. MÉD.UIS.2020;34(1):101-6

Pénfigo sifilítico: reporte de un caso

RESUMEN La sífilis congénita (SC) es el resultado de la infección producida por el Treponema pallidum al feto, en una madre con sífilis gestacional (SG). La enfermedad puede ser asintomática en el 60% de los recién nacidos (RN) afectados, siendo además difícil de diagnosticar clínica e histológicamente, porque tanto sus patrones clínicos como histológicos, pueden imitar otras enfermedades dermatológicas del RN. Siendo la SC una enfermedad prevenible, siguen presentándose casos con manifestaciones clínicas severas. Presentamos el caso de un RN con SC de presentación grave desde el nacimiento, con manifestaciones cutáneas que incluyeron múltiples lesiones tipo vesículas y bulas, con zonas hemorrágicas y áreas denudadas con maceración, descamación y costras, de predominio en palmas y plantas, compatibles con pénfigo sifilítico. Reconocer las diversas manifestaciones clínicas, en especial las cutáneas, es fundamental para realizar un diagnóstico oportuno de SC en RN y lactantes.

ABSTRACT Congenital syphilis (CS) is the result of an fetal infection caused by Treponema pallidum, in a mother with gestational syphilis (GS). The disease can be asymptomatic in 60% of affected newborns (NBs), and is also difficult to diagnose clinically and histologically, because both its clinical and histological patterns can mimic other dermatological diseases of the NB. Since CS is a preventable disease, cases with severe clinical manifestations continue to appear. We present the case of an NB with severe CS presenting from birth, with skin manifestations that included multiple vesicle and bulla-like lesions, with hemorrhagic areas and denuded areas with maceration, scaling and scabs, predominantly on the palms and soles, compatible with syphilitic pemphigus. Recognizing the various clinical manifestations, especially skin manifestations, in this disease, is essential to make a timely diagnosis of CS in newborns and infants.

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.

Pénfigo foliáceo de presentación inusual / Penphigus foliaceus of unusual presentation

Si bien las enfermedades ampollares autoinmunes son infrecuentes en la infancia y la juventud, es importante tenerlas en cuenta dentro del diagnóstico diferencial, junto con otras enfermedades inflamatorias o infecciosas de mayor prevalencia, para lograr un diagnóstico temprano y así indicar el tratamiento preciso y oportuno. El pénfigo foliáceo es una enfermedad ampollar poco común, salvo en la zona endémica de Brasil. Se presenta un caso de pénfigo foliáceo con eritrodermia como cuadro clínico con mala evolución (AU)

Although autoimmune bullous diseases are infrequent in childhood and youth, it is important to take them into account within the differential diagnosis, along with other more prevalent inflammatory or infectious diseases, to achieve an early diagnosis and thus indicate precise and timely treatment. Pemphigus foliaceus is a rare autoimmune bullous diseases, except in the endemic area of Brazil. This case report presents a patient with the unusual erythrodermic presentation of pemphigus with poor evolution (AU)

Características clínicas e histopatológicas de los pacientes con enfermedades ampollares autoinmunes: estudio retrospectivo en el Hospital Privado Universitario de Córdoba / Clinical and histopathological characteristics of autoimmune blister diseases: a retrospective study in Private University Hospital of Córdoba

RESUMEN Las enfermedades ampollares autoinmunes (EAA) comprenden un conjunto heterogéneo de patologías de baja frecuencia de presentación con evolución crónica, remisiones, exacerbaciones y una importante morbimortalidad aun con tratamiento temprano y adecuado. Existen pocos estudios epidemiológicos en nuestra población que incluyan todo el espectro de enfermedades ampollares. Objetivos : Determinar las características clínico-epidemiológicas de las EAA, describir tratamientos realizados, tiempo de control de actividad de la enfermedad y tiempo desde iniciado el tratamiento hasta la remisión completa. Determinar recaídas de las patologías estudiadas, evaluando si existe relación entre las mismas y algún factor reconocido. Materiales y métodos : Estudio retrospectivo y descriptivo. Se incluyeron todos los pacientes con diagnóstico clínico, histopatológico y de inmunofluorescencia de las EAA que hayan tenido seguimiento por al menos un año en el servicio de dermatología del HPUC, en el período de enero de 2008 hasta noviembre de 2018. Resultados : Se incluyeron 53 pacientes de los cuales el 50,9% tenía Penfigoide Ampollar (PA), 22,6% Dermatitis Herpetiforme (DH) y 17% de Pénfigo vulgar (PV). La edad de los pacientes se encontró entre 21 y 90 años. Las ampollas tensas y erosiones fueron las presentaciones más comunes. Los tratamientos sistémicos se realizaron en el 74% de los casos. Registramos recaídas en el 63% de los casos y dos casos de muerte durante el tratamiento. Se estableció más frecuencia de enfermedad cardiovascular en los pacientes con PA. Conclusiones : Es el primer trabajo retrospectivo sobre EAA en nuestro medio. Nuestros hallazgos permiten determinar una frecuencia importante de PA en la consulta dermatológica y de DH, hecho no reportado en otros estudios con poblaciones similares.

ABSTRACT The autoimmune bullous diseases (ASD) comprise a heterogeneous group of pathologies with a low frequency of presentation that presents a chronic evolution, with remissions, exacerbations and an important morbidity and mortality even with early and adequate treatment. There are few epidemiological studies in our population that include the entire spectrum of bullous diseases. Objectives : To determine the clinical-epidemiological characteristics of AAS, describe treatments performed, control time of disease activity and time from treatment initiation to complete remission. Determine relapses of the studied pathologies, evaluating if there is a relationship between them and some recognized factor. Materials and methods : Retrospective and descriptive study. All patients with clinical, histopathological and immunofluorescence diagnosis of the AAS who had followed up for at least one year in the dermatology service of the HPUC, from January 2008 to November 2018 were included. Results: 53 were included. patients of which 50.9% had Bullous Pemphigoid (BP), 22.6% Dermatitis herpetiformis (DH) and 17% Pemphigus Vulgaris (PV). The age of the patients was between 21 and 90 years. Tense blistering forms and erosions were the most common forms of presentation. Systemic treatments were performed in 74% of the cases. We recorded relapses in 63% of the cases and two cases of death during the treatment. More frequency of cardiovascular disease was established in patients with BP. Conclusions : It is the first retrospective work on EAA in our environment. Our findings allow determining an important frequency of PA in the dermatological consultation and DH, fact this last, not reported in other studies with similar populations.

Enfermedad de Hailey-Hailey, una familia diagnosticada a partir de un caso propositus / Hailey-Hailey diseases, a family diagnosed starting from a case propositus

RESUMEN Introducción: La enfermedad de Hailey-Hailey, también conocida como pénfigo benigno familiar, es una enfermedad de muy baja frecuencia de aparición, aunque es una genodermatosis,las manifestaciones clínicas se manifiestan en la adolescencia o adultez temprana. Objetivo: Profundizar en los elementos que permiten el diagnóstico temprano de la enfermedad de Hailey-Hailey. Presentación del caso: En Las Tunas, provincia oriental de Cuba, es atendida en consulta especializada multidisciplinaria de genodermatosis, una mujer de 50 años de edad, quien presentabalesiones eritematosas, vesiculosas y erosivas, localizadas en zonas de pliegues, que habían aparecido desde la adolescencia, siendo tratadas en varias ocasiones como una micosis superficialo dermatitis. Se lerealiza estudio histopatológico que constató el diagnósticode enfermedad de Hailey-Hailey. Se estudiaron los demás miembros de la familia afectados con similareslesiones dermatológicasy se corrobora el diagnóstico familiar. La paciente fue tratada con esteroides en dosis antinflamatorias, vitaminoterapia y terapéutica tópica consistente en uso de fomentos antisépticos naturales y crema antinflamatoria de aloe, con buena respuesta a la terapéutica. Conclusiones: Se presenta el caso porque la enfermedad de Hailey Hailey es infrecuente, las manifestaciones comienzan en la adolescencia, pudiendo confundirse con otras dermatosis. En la investigación se determinó el diagnóstico de los demás miembros de la familia afectados a partir del caso propositus.

ABSTRACT Introduction: The Hailey-Hailey diseases,also known asbenign familial pemphigus, is an disease of very low appearance frequency,although it is a genodermatoses, the clinical manifestations are manifested in the adolescence or early adulthood. Objective: To deepen in the elements that allows the early diagnosis of Hailey-Hailey diseases. Case presentation: In Las Tunas, oriental county of Cuba, is assisted in multidisciplinary specialized consultation of genodermatoses, a 50-year-old woman who presented lesions erytmematous, vesiculous and erosive, located in areas of pleats that had appeared from the adolescence, being treated in several occasions like a superficial mycosis or dermatitis. She is carried out study histopathologyc that verified the diagnosis of Hailey-Hailey diseases. The other members of the family were studied affected with similar injure dermatological and the family diagnosis is corroborated. The patient was treated with steroids in dose antinflammatory, vitamintherapy and topical therapeutic consistentin use of antiseptic natural foments and cream antinflammatory of aloe, with good answer to the therapy. Conclusions: The case is presented because the Hailey-Hailey diseases areuncommon; the manifestations begin in the adolescence, being able to make a mistake with other dermatomes.In the investigation the diagnosis of the other members of the family was determined affected starting from the case propositus.