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Pemphigus vulgaris is a blistering disease of autoimmune origin that affects skin and mucous membranes, mediated by anti-IgG autoantibodies directed against the autoantigens Dsg 1 and 3 present in keratinocytes. It causes a loss of cell adhesion just above the basal layer, forming intraepidermal blisters. We describe the case of a 14-year-old adolescent with no significant medical history, who presented with a bilateral and symmetrical generalized dermatosis on the oral mucosa, characterized by flaccid blisters, some of which were ulcerated and had hematomas on the surface, and a positive Nikolsky sign, painful for 1 month. A skin biopsy was performed, which revealed a suprabasal intraepidermal acantholytic blister and mild superficial perivascular lymphocytic inflammatory infiltrate in the dermis. Treatment was initiated with Wondra's magistral solution, topical and systemic steroids, and immunosuppressants with a good response. This case is reported because pemphigus vulgaris is an uncommon disease in adolescence, with its peak incidence between the fourth and sixth decades of life. [ABSTRACT FROM AUTHOR]

Pemphigus erythematosus (PE) is a rare disorder that belongs to a group of chronic autoimmune blistering diseases. It is a form of pemphigus with an overlapping clinical and immunological presentation of pemphigus foliaceus and lupus erythematosus, mediated by autoantibodies targeting desmoglein 1. It has a broad clinical spectrum, and severe cases can result in multi-organ compromise. To date, there is no established treatment for severe cases of PE. We describe a case of a previously healthy patient with a 4-month clinical course of generalized painful erythematous desquamative plaques and blisters, associated with a 10 kg weight loss. The clinical and histopathological findings were consistent with PE. An adjusted rituximab protocol was used, achieving disease remission within 20 days. Rituximab is the mainstay of therapy and a well-known effective treatment for pemphigus vulgaris with a prolonged remission; nevertheless, there are no established protocols and limited evidence in PE. [ABSTRACT FROM AUTHOR]

Introduction: Pemphigus are rare, but potentially serious, autoimmune blistering diseases that can be associated with catastrophic consequences if an appropriate diagnostic and therapeutic approach is not performed. The Dermatology Service of the University of Antioquia at its main headquarters, Hospital Alma Mater de Antioquia, and Hospital Universitario San Vicente Fundación, is a reference center for the study and management of these diseases. Methodology: review of the literature and socialization in the group of teachers and residents of the University of Antioquia to develop management guidelines. Results: Guidelines are described in the diagnosis and treatment of patients with pemphigus vulgaris and pemphigus foliaceus. Likewise, a narrative review of the scientific evidence that supports what is recommended in this article is carried out. [ABSTRACT FROM AUTHOR]

Senear-Usher syndrome is a localized variant of pemphigus foliaceus, rare, with a chronic course, manifested by well-defined erythematous squamous plaques, with a seborrheic appearance and distribution, which adopts a butterfly-wing pattern resembling cutaneous lupus erythematosus (characteristic in our case) and a positive Nikolsky's sign. With the presence of fragile and fleeting blisters, it does not affect the mucosa, it is a disease of low incidence and represents 0.5 to 3.2 cases per 100,000 inhabitants per year. In ages from 15 to 84 years, on average at 54 years, without predominance of race or sex. We present the case of a 31-year-old male patient, skin phototype V/VI according to Fitzpatrick, with no signifi- cant history, presenting bilateral and symmetrical generalized dermatosis beginning in the central facial region characterized by well-defined erythematous plaques with regular borders, with presence of thick oily yellow scales and bruises on the surface, which spread to other seborrheic areas of the chest, anterior and posterior, exulcerated areas, hyperpigmented macules of variable sizes, pruritic with a 1-year history. A skin biopsy was performed with findings consistent with this disease. Treatment with topical and systemic steroids and immunosuppressants was started with a good response. A case is reported because it is a rare variant of pemphigus foliaceus, so it is important to report this case in Venezuela. [ABSTRACT FROM AUTHOR]

A histoplasmose, causada pelo fungo Histoplasma capsulatum, é uma micose endêmica que eventualmente pode resultar em doença grave. A maioria dos pacientes que desenvolve histoplasmose severa é imunodeprimida ou está em extremos de idade. Pode apresentar-se clinicamente com sintomas constitucionais, linfadenopatia, hepatoesplenomegalia, lesões de pele e mucosas. O diagnóstico é feito através de suspeita clínica associada à visualização direta/cultura do fungo em tecidos e/ou exame histopatológico. Anticorpos anti Histoplasma e antígeno urinário podem auxiliar na investigação. Paciente masculino, 55 anos, agricultor, encaminhado da atenção primária por perda de peso e placa eritematosa e dolorosa em bordo de língua iniciados há 5 meses. Realizada biópsia da lesão, sem identificação de microrganismos em exames diretos e culturas. Após 4 meses evoluiu com disfonia e lesões de pele bolhosas, frágeis, pruriginosas, com ulcerações, crostas e outras alterações tróficas crônicas, com saída de secreção serosa. As lesões iniciaram em membros superiores e apresentaram disseminação para todo o corpo. Foi submetido à biópsia de pele com evidência de Histoplasma capsulatum em exame micológico direto. Realizada, então, internação hospitalar para tratamento intravenoso com anfotericina B devido à grande extensão das lesões. Foi realizada ampla investigação de imunodeficiências, sem evidência de neoplasias e outras doenças. Sorologias para HIV, hepatites e sífilis não reagentes, VHS elevado, demais exames sem alterações. Durante o tratamento antifúngico as lesões apresentaram importante melhora, com evolução para crostas e redução progressiva da saída de secreção até interrupção completa. O paciente recebeu alta com itraconazol 400 mg/dia após uso de 10 dias de anfotericina B complexo lipídico. Em consulta ambulatorial de retorno mantinha lesões crostosas muito pruriginosas, com algumas lesões bolhosas. Foi obtido o resultado do exame anatomopatológico de língua e pele com diagnóstico de pênfigo vulgar, prescrita corticoterapia com prednisona 1 mg/kg/dia (dose reduzida posteriormente) e mantido itraconazol. Com o tratamento antifúngico para histoplasmose cutânea associado à corticoterapia para tratamento de pênfigo vulgar o paciente evoluiu com cicatrização completa das lesões de pele e mucosa. Devido à sua endemicidade, infecções fúngicas devem ser investigadas em pacientes com lesões de pele e sintomas constitucionais, mesmo na ausência de imunodeficiências conhecidas.

Pemphigus are a group of autoimmune diseases characterized by the production of autoantibodies against desmosome antigens, especially desmogleins 1 and 3. They manifest with intraepidermal blisters on the skin and mucous membranes, caused by the separation of epidermal cells by a process called acantholysis. Diagnosis is made based on clinical manifestations and is confirmed by histological, serological and immunofluorescence tests. Corticosteroids and immunosuppressive agents have been the cornerstone of treatment against pemphigus vulgaris and pemphigus foliaceus for several decades, this has improved the prognosis of patients; however, mortality remains high since this therapy can cause prolonged suppression of the immune system and may cause catastrophic side effects. Recently, the therapeutic approach has revolved around specific immunosuppressive strategies such as rituximab, an anti-CD20 monoclonal antibody. Current evidence indicates that its efficacy is superior when administered early in the course of the disease, which is why it should be used as first-line medication in moderate and severe forms of the disease to increase efficacy and improve patient prognosis. This review focuses on the current treatment of pemphigus, based on the most up to date literature such as the guidelines for the management of pemphigus vulgaris and foliaceus of the European Academy of Dermatology and Venereology, as well as other articles relevant to the subject. [ABSTRACT FROM AUTHOR]

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Antecedentes: Dentro de las enfermedades autoinmunes que provocan lesiones erosivas y ampollas en la piel y las mucosas se encuentra el pénfigo. Un subtipo raro de esta enfermedad es el pénfigo seborreico, o síndrome de Senar-Usher, caracterizado por ampollas rotas y costras que afectan las áreas corporales que secretan grasa. Reporte de caso: Paciente femenina de 40 años, atendida inicialmente en una unidad de primer nivel por un cuadro de 45 días de evolución, caracterizado por lesiones costrosas gruesas de base eritematosa, pruriginosas y dolorosas, de localización centro-facial, con posterior extensión hacia el abdomen, tórax y extremidades. El tratamiento consistió en prednisolona, con evolución favorable. La biopsia de las lesiones confirmó el diagnóstico de pénfigo seborreico. Conclusión: El síndrome de Senear-Usher, o pénfigo seborreico, es una enfermedad excepcional, de origen multifactorial. El diagnóstico oportuno y tratamiento adecuado son factores decisivos para evitar la evolución de la enfermedad a formas avanzadas.

Background: Among the autoimmune diseases causing erosive lesions and blisters on skin and mucous membranes is pemphigus. Within this is a rare subtype known as seborrheic pemphigus or Senear-usher syndrome which is characterized by broken blisters and crusts involving the seborrheic areas. Case report: A 40-year-old female patient, initially treated in a first level unit for a condition of 45 days of evolution, characterized by thick scabby lesions with an erythematous base, pruritic and painful, located in the center of the face, with posterior extension towards the abdomen, thorax, and extremities. Treatment consisted of prednisolone, with favorable evolution. The biopsy of the lesions with the diagnosis of seborrheic pemphigus. Conclusion: Senear-usher syndrome is a rare disease of multifactorial origin. Early diagnosis and adequate treatment are decisive factors to avoid the evolution and advanced forms of the disease. [ABSTRACT FROM AUTHOR]

BACKGROUND: Pemphigus vulgaris is an autoimmune mucocutaneous disease resulting from the production of antidesmogleins against epidermal desmosomes. These autoantibodies produce blisters by acantholysis. Although pemphigus vulgaris frequently maintains its clinical expression, there is a poorly recognized percentage of patients that present alterations in their autoimmune profile and develop pemphigus foliaceous. CLINICAL CASE: A 25-year-old female patient with a 3-year history of pemphigus vulgaris in remission, who presented plaques with white oily scale on an erythematous base in seborrheic topography. The histopathological diagnosis was pemphigus foliaceus that showed clinical and histopathological transition from pemphigus vulgaris to pemphigus foliaceus. CONCLUSIONS: The clinical transition from pemphigus vulgaris to pemphigus foliaceus is a rare dermatological process of unknown etiology that correlates with the transition of anti-desmoglein 1 to anti-desmoglein 3 autoantibodies. Currently, information on this transition in Latin American patients is limited and highlights the importance of keeping the patient under surveillance. [ABSTRACT FROM AUTHOR]

Copyright of Ginecología y Obstetricia de México is the property of Federacion Mexicana de Ginecologia y Obstetricia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: IgA pemphigus is a rare, chronic bullous disease characterized by the presence of IgA autoantibodies against desmosomes. Two main variants are recognized: the subcorneal pustular dermatosis (SCD) type and intraepidermal neutrophilic dermatosis (NIE). The diagnosis is based on the clinical features and is confirmed by histology and the presence of IgA deposits between the keratinocytes. Dapsone is the first-line treatment. In this report, we presented a patient with the intraepidermal neutrophilic dermatosis variant who initially responded to oral corticosteroids and dapsone. The purpose of this report is to briefly review the most important features of this rare entity. [ABSTRACT FROM AUTHOR]

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.

Introducción: el pénfigo IgA es una enfermedad ampollosa autoinmune que se caracteriza por la presencia de lesiones pustulosas y ampollosas asociadas a dolor y prurito. Si bien, existen dos tipos principales: la dermatosis IgA neutrofílica intraepidérmica y la dermatos is pustulosa subcórnea, el amplio espectro en la presentación clínica y la superposición de los hallazgos con otras enfermedades ampollosas, hace difícil el enfoque diagnóstico y el tratamiento. Caso clínico: en el presente reporte de caso se describe a una mujer de 50 años de edad con una presentación atípica de enfermedad ampollosa la cual fue clasificada finalmente como pénfigo IgA con características de superposición, acorde a los hallazgos clínicos y los resultados de la inmunofluorescencia directa. Conclusiones: el pénfigo IgA actualmente es considerado un espectro de enfermedades ampollosas en el cual puede existir superposición de sus características con implicaciones terapéuticas y en el pronóstico que deben ser tenidas en cuenta.

Pemphigus vulgaris (PV) is a dermatosis that affects the skin and mucous membranes. Its incidence is two new cases/million inhabitants/year in Central Europe. It is known to be multifactorial and involves interaction between genetic predisposition and environmental factors. This study reports a rare case of PV after plastic surgery. We present the case of a 51-year-old woman with suture dehiscence in the postoperative period of mammoplasty with prosthesis and abdominoplasty and, subsequently, the appearance of bullous lesions in the scars. These lesions were later diagnosed as PV. The patient evolved with improvement of the lesions after corticosteroid therapy. It is also noteworthy that the patient had COVID-19 during hospitalization, a known factor of exacerbation of pemphigus, but without worsening of the lesions and with complete regression of the infectious condition after treatment. The clinical evolution of PV can be limited to mucosal involvement or progress to the skin. Large eroded, exudative, bleed ing, confluent, painful areas are formed, with no tendency to heal 1 . Cutaneous involvement can occur anywhere, being more common on the scalp, trunk, abdomen and flexures. [ABSTRACT FROM AUTHOR]

Background: Pemphigus vulgaris comprises a group of heterogeneous blistering autoimmune diseases of the skin and mucosa. Esophageal involvement within pemphigus vulgaris is rare with an uncertain prevalence that requires a detailed diagnostic and a therapeutic approach. Clinical case: 37-year-old female, with a history of treatment with Cox-2 inhibitors due to herniated disc. She is sent to the Gastroenterology Service for weight loss of approximately 5 kilos in a month, with the presence of dysphagia, odynophagia and retrosternal pain with poor tolerance to the oral route. Endoscopy was performed, which reported esophagitis dissecans superficialis (EDS), erythematous gastropathy of the antrum and normal duodenum. Findings were correlated with the diagnosis of pemphigus vulgaris with exclusive involvement of the esophagus. The evaluation did not identify lesions on the skin, oral cavity or other mucous membranes. A new endoscopy was performed as a control and it was found immunofluorescence of the esophageal biopsy reactive to IgG 2. Initial management was given with glucocorticoids, anti-inflammatories and immunosuppressants. Conclusions: The importance of the study of pemphigus lies not only in the high associated morbidity and mortality, but also in its intrinsic rarity and the complexity of its detection, given that patients usually take several months to have an accurate diagnosis and even more time to achieve therapeutic goals. It is a priority the dissemination of the study of pemphigus among health professionals involved in its detection. [ABSTRACT FROM AUTHOR]

Antecedentes: El pénfigo es una enfermedad autoinmune crónica y recalcitrante que afecta piel y mucosas. El tratamiento de primera línea son los corticosteroides sistémicos, aunque hay pacientes refractarios a estos y a otros inmunosupresores. Como alternativa, desde el año 2000 se ha utilizado el rituximab con buenos resultados, pero sin información de su comportamiento en población mexicana. Objetivo: Evaluar la respuesta clínica al tratamiento con rituximab en pacientes mexicanos con pénfigo. Métodos: Se trató de un estudio transversal, retrospectivo en un hospital de tercer nivel, en el que se incluyeron pacientes con pénfigo moderado-severo tratados con rituximab entre 2007 y 2020. Resultados: Se obtuvieron seis expedientes de pacientes con diagnóstico de pénfigo, cuatro presentaron la variedad vulgar y dos, la variedad foliácea; todos recibieron tratamiento sistémico inmunosupresor previo al rituximab. Los seis pacientes tuvieron remisión de la enfermedad en un promedio de 12.5 semanas. Conclusión: El rituximab para el tratamiento de pacientes con pénfigo moderado-severo refractario a tratamiento inmunosupresor demostró ser de gran utilidad y logró un control de la enfermedad a mediano plazo, sin efectos adversos severos ni idiosincráticos en la población mexicana estudiada.

Introdução: O pênfigo vulgar (PV) é uma doença autoimune caracterizada pela formação de bolhas em mucosas e/ou pele que estouram facilmente, gerando lesões irregulares. O prognóstico depende de um tratamento precoce, reduzindo riscos de infecção secundária e/ou agravamento da doença que pode levar ao óbito. Objetivo: Relatar um caso clínico e sua condução com discussão pautada nos aspectos atuais da literatura sobre a doença com relação à abordagem odontológica. Relato de Caso: Paciente do sexo feminino, 24 anos, leucoderma, com queixa de dor e descamação gengival que não regrediu após 1 ano de terapias focadas em controle de biofilme e hábitos de higiene bucal. Ao exame extrabucal não foram observadas lesões. Ao exame intrabucal observou-se a ocorrência de eritema em gengiva marginal e papilar por vestibular com pequenos pontos de ulceração rasa e dolorosa. O teste de Nikolsky realizado com o auxílio de uma seringa resultou em positivo. Realizou-se na sequência uma biópsia incisional na região próxima a este local. A histopatologia mostrou lesão de mucosa por acantólise, com formação de fenda intraepitelial e células de Tzank, culminando no diagnóstico de PV. O tratamento consistiu em corticosteroides tópicos, principalmente às custas de Clobetasol em terapia oclusiva, mostrando adequado controle de úlceras e dor. Conclusão: Quadros de gengivite não associadas ao biofilme representam desafio na rotina do cirurgião-dentista, e o PV deve ser parte do rol de hipóteses para o desenvolvimento de uma propedêutica adequada, proporcionando qualidade de vida e diagnóstico precoce.

Pemphigus foliaceus (PF) is part of a group of rare and serious autoimmune diseases, which is mediated by autoantibodies directed against cellular adhesion proteins present in the skin and is characterized histologically by acantholysis and clinically by the presence of blisters and cutaneous crusts. We report a case of PF in a 22-year-old woman presenting with widespread blisters and crusts, without mucosal involvement, and a sui generis smell. The diagnosis is later confirmed by the findings of histopathology and direct immunofluorescence and, due to demographic characteristics, endemic PF or fogo selvagem is inferred. [ABSTRACT FROM AUTHOR]

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A Síndrome da Hiperestesia Felina (FHS) é uma condição clínica, caracterizada por contrações musculares involuntárias e mudanças de comportamento. É uma síndrome rara, muitas vezes de origem desconhecida, sendo que as causas mais comuns associadas são doenças relacionadas à pele, tais como dermatites bacterianas, fúngicas, alérgicas e doenças imunomediadas. Causas relacionadas ao sistema nervoso central e distúrbios comportamentais também são relatadas. Os sinais clínicos são variados, o diagnóstico muitas vezes é complexo e o tratamento varia de acordo com a causa de base. O objetivo deste relato de caso é descrever um caso de FHS de possível origem dermatológica. Um felino, macho, de três anos, da raça Siamês, foi atendido apresentando doença do trato urinário inferior felino há nove dias. Associado, o animal possuía áreas eritematosas e de hipotricose por toda a face, principalmente ao redor dos olhos, nariz e pavilhões auriculares, juntamente com hipersensibilidade dolorosa ao toque e fasciculações cutâneas na região lombar, coccígea e membros pélvicos. Foi realizado exame histopatológico das lesões de pele, onde foi visualizada dermatite superficial perivascular crônica proliferativa. Com base nos achados de exame físico e nos exames complementares, o animal foi diagnosticado com síndrome da hiperestesia felina secundária à dermatopatia.

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. ABSTRACT 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. RESUMEN 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.

Background: Pemphigus (Pm) is a chronic and recalcitrant autoimmune disease that affects the skin and mucous membranes. The first line of treatment are systemic corticosteroids; however, there are patients who are refractory to them, as well as to other immunosuppressants. Rituximab has been used as a successful alternative since 2000 with good results but without information on its behavior in the Mexican population. Objective: To assess the clinical response to treatment with rituximab in Mexican patients with pemphigus. Methods: This was a cross-sectional, retrospective study in a tertiary hospital, which included patients with moderate-severe pemphigus who had been treated with rituximab from 2007 to 2020. Results: Six medical records of patients diagnosed with pemphigus were obtained; four of them with Pm vulgaris, and two of them with pemphigus foliaceus; all patients had received systemic immunosuppressive therapy prior to rituximab. All six patients went into remission of the disease in an average of 12.5 weeks. Conclusion: The use of rituximab for the treatment of patients with moderate-severe Pm who were refractory to immunosuppressive therapy proved to be very useful, and control of the disease was achieved in the medium term, without severe or idiosyncratic adverse effects in the analyzed Mexican population. [ABSTRACT FROM AUTHOR]

Feline Hyperesthesia Syndrome (FHS) is a clinical condition, characterized by involuntary muscle contractions and changes in behavior. It is a rare syndrome, often of unknown origin, and the most common associated causes are skin related disorders, such as bacterial, fungal dermatitis, allergics and immune-mediated diseases. Causes related to the central nervous system and behavioral disorders are also reported. The clinical signs are varied, the diagnosis is often complex and the treatment varies according to the underlying cause. The purpose of this case report is to describe a case of FHS of possible dermatological etiology. A three-year-old Siamese male feline was treated for feline lower urinary tract disease throughout the duration of nine days. Associated, the animal also presented erythematous areas and hypotrichosis all over the face, especially around the eyes, nose and auricular pavilions, along with painful hypersensitivity to the touch and skin fasciculations in the lumbar and coccygeal region and pelvic limbs. An histopathological exam of the skin lesions was performed, where a chronic, superficial perivascular dermatitis was seen to be proliferative. Based on physical examination findings and additional tests, the animal was diagnosed with feline hyperesthesia syndrome secondary to dermatopathy. [ABSTRACT FROM AUTHOR]

Introducción y objetivo: Las manifestaciones dermatológicas son frecuentes en las enfermedades autoinmunes, sin embargo la proporción de presentación varía dependiendo de la enfermedad y de factores genéticos, epigenéticos y ambientales. En algunas enfermedades autoinmunes se ha estudiado ampliamente el compromiso cutáneo como un órgano afectado secundariamente por la enfermedad, mientras que en otras el órgano blanco, afectado primariamente, es la piel. No hay evidencia conocida de las manifestaciones dermatológicas agrupadas en un solo estudio. Por lo tanto, en este estudio se describen y clasifican las manifestaciones dermatológicas de las enfermedades autoinmunes. Métodos: Estudio observacional, descriptivo de corte transversal retrospectivo. La población estudio fueron pacientes con enfermedades autoinmunes con manifestaciones dermatológicas en un hospital universitario de Colombia entre los años 2012-2016 en pacientes mayores de 13 años. Resultados: Se analizaron 1.037 pacientes con hallazgos de alguna manifestación dermatológica. En artritis reumatoide hasta en el 85% de los pacientes, psoriasis 99%, púrpura trombocitopenica inmune 40%, lupus eritematoso sistémico 53%, vitíligo 100%, síndrome de Sjögren 96%, anemia hemolítica autoinmune 83%, esclerosis sistémica 65%, pénfigo 76%, polimiositis 69%, síndromes vasculíticos 67% y dermatomiositis 86%. Conclusiones: Se reconoce la variedad de manifestaciones dermatológicas que pueden ser encontradas, sin embargo estas dependen de cada tipo de enfermedad, no pudiéndose establecer una misma frecuencia para ellas en total por el compromiso sistémico o específico según su clasificación.

Background: For the most part, adverse reactions caused by subcutaneous allergen-specific immunotherapy (AIT) are directly induced by the administration of an allergen; there is usually redness and induration at the area of the injection. Other skin lesions are extremely unusual. Case report: A 40-year-old woman with allergic rhinoconjunctivitis and asthma due to sensitization to grass pollen had received monthly doses of 0.5mL of a subcutaneous polymerized extract of grass pollen with glutaraldehyde and adsorbed in aluminum hydroxide. Five hours after the seventh dose, the patient presented stinging and itching at the area of the injection of the immunotherapy, together with a blistering reaction that appeared later. Conclusion: Although adverse reactions by allergen-specific immunotherapy are rare and difficult to verify, it is important to perform the necessary analyses to identify the causes or their relation to the symptoms to avoid reactions in the future. [ABSTRACT FROM AUTHOR]

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Pemphigus is an autoimmune and chronic disease with low incidence and prevalence. Nevertheless it could reach a 75% mortality rate without treatment. There are two principal types: vulgar pemphigus and foliaceus pemphigus, which are clinically characterized by skin blister appearance, with mucosal affection in patients with pemphigus vulgaris. This review details the epidemiology, etiology, physiopathology and treatment of this disease, remarking the steroid importance as the cornerstone in the management of these patients. [ABSTRACT FROM AUTHOR]

Introducción: el pénfigo vulgar es una enfermedad autoinmune poco común de piel y mucosas, su etiología es desconocida y presenta una elevada tasa de mortalidad. Las manifestaciones de esta patología son comunes en cavidad bucal; por ende, el odontólogo tiene una gran responsabilidad en realizar un diagnóstico oportuno y, de esta manera, mejorar el pronóstico. Presentación del caso: paciente sexo femenino de 63 años con diagnóstico de pénfigo vulgar y lesiones en cavidad bucal y piel; el tratamiento se realizó en equipo entre el departamento de estomatología y dermatología. El manejo sistémico fue con prednisolona en dosis altas que disminuían en periodos determinados; además, se medicó con inmunosupresor, antihistamínico, antibióticos y antimicóticos. Para tratar las lesiones en cavidad bucal se utiliza una fórmula magistral compuesta por hidrocortisona, vitamina A, E, glicerina de bajo peso molecular y xilocaína en orabase aplicada en las lesiones. El tratamiento se realizó por 4 meses, a la primera semana se logró la resolución de la sintomatología en cavidad bucal y piel; se logró la cicatrización completa de todas las lesiones y no se registró recidiva luego de culminado el tratamiento. Conclusión: la literatura coindice en que la prednisolona es el medicamento de elección para tratar dicha patología, se encuentra que un diagnóstico oportuno evitaría el uso de estos medicamentos en altas dosis y por largos periodos, evitando así reacciones adversas; el uso de la fórmula magistral para lesiones en cavidad bucal no se reporta en la literatura, por ende, se considera importante su divulgación.

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

Introducción y objetivo: El pénfigo es una enfermedad autoinmune dermatológica que produce lesiones ampollosas por la acción de anticuerpos contra proteínas en los desmosomas, provocando pérdida de la cohesión de las células epidérmicas, generando múltiples manifestaciones dermatológicas. El objetivo es describir una serie de pacientes con pénfigo valorados por dermatología. Métodos: Estudio observacional, descriptivo de corte transversal retrospectivo. La población estudio correspondió a pacientes mayores de 13 años con pénfigo, atendidos en la consulta dermatológica en un hospital universitario de alta complejidad entre los años 2012-2016. Resultados: Se analizó un total de 25 pacientes con promedio de edad de 41,1 años, mediada de tiempo de evolución de 1 año, el 60% fueron hombres. El 76,7% procedían del área rural. El 40% ingresó por consulta externa, mientras que el 26,7% por urgencias; y el 33,3% ingresó tanto por consulta externa como por urgencias. Dentro de las características clínicas presentaron afección de piel en el 96%, compromiso de la mucosa el 52%, lesiones en cuero cabelludo 24%, presencia de ampollas 76%, presencia de ampollas pequeñas 56%, presencia de ampollas superficiales 64%, eritema 60%, dolor 44%, signo de Nikolsky 36%, ulceras 52%, presencia de pénfigo vulgar 88%, pénfigo violáceo 4%, otros pénfigos 8%, enfermedad de Hailey-Hailey 4%. Conclusiones: Las manifestaciones dermatológicas son múltiples y se puede encontrar afección mucosa en gran cantidad de los pacientes, los hallazgos se relacionan con datos de publicaciones internacionales y pueden encontrarse presentaciones atípicas de la enfermedad.

Introducción y Objetivo: La enfermedades autoinmunes están relacionadas con alteraciones en la inmunidad que se presentan con mayor frecuencia en población joven, no existe evidencia clara sobre las enfermedades autoinmunes en los adultos mayores, debido a que prevalecen otras enfermedades que pudieran estar relacionadas con el proceso de envejecimiento. Nuestro objetivo es realizar una descripción de las enfermedades autoinmunes en pacientes geriátricos del Hospital Universitario de Santander, Colombia. Métodos: Estudio observacional, descriptivo de corte transversal retrospectivo, la población de estudio fue pacientes con enfermedades autoinmunes mayores de 65 años en el Hospital Universitario de Santander entre el 2012 y el 2016. Resultados: Se analizó una muestra de 1.463 pacientes con enfermedades autoinmunes, se obtuvo una muestra de 236 pacientes mayores de 65 años para su estudio, con un promedio de edad de 72 años, dentro de las enfermedades autoinmunes la población geriátrica ocupa un 16.13%, la enfermedad autoinmune más prevalente en esta población fue la artritis reumatoide con 94 casos que equivalen casi al 50% de los pacientes mayores con enfermedades autoinmunes, psoriasis 13%, purpura trombocitopenica inmune 8%, esclerosis sistémica 6%, vitíligo 5%, síndrome de Sjögren 4%, síndrome de Guillain-Barré 4%, pénfigo 4%, colitis ulcerativa 3%, anemia hemolítica autoinmune 3%, lupus eritematoso sistémico 2%, enfermedad de Graves 2% y otras enfermedades corresponden a menos del 2% cada una. CONCLUSIONES: Las enfermedades autoinmunes a pesar de ser más frecuentes en la población joven, se encuentra presente en la población adulta mayor, siendo más frecuente la artritis reumatoide que prevalece sobre las otras enfermedades.

Copyright of RGO: Revista Gaúcha de Odontologia is the property of RGO: Revista Gaucha de Odontologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

In autoimmune diseases, there is loss of intercellular adhesion and alteration of the skin barrier, so that moderate to severe forms require hospital management. There are no reports on the characteristics of the patients hospitalized by these entities in the Colombian populationThe objective of this study was to describe the epidemiological and clinical characteristics of patients hospitalized for pemphigus vulgaris, pemphigus foliaceus or bullous pemphigoid treated by the Sección de Dermatología, Universidad de Antioquia at the Hospital Universitario San Vicente Fundación. We included 99 cases, the most frequent disease being pemphigus foliaceus. Most cases were classified as moderate to severe and treatment was based on systemic steroids and azathioprine as a steroid saver. The mean hospitalization was 50.8 days for pemphigus vulgaris, 48.4 days for pemphigus foliaceus, and 39.3 days for pemphigoid blisters. The most frequent complications were infections. These diseases require a careful approach to the complications inherent to the pathology or its treatment. They entail prolonged hospitalizations and long-term therapeutic support to avoid relapses and hospital readmissions. [ABSTRACT FROM AUTHOR]

Propuesta de reconocimiento del estándar de comodidad en clientes con pénfigo vulgar utilizando la Lógica Fuzzy O objetivo é propor a Lógica Fuzzy para reconhecimento de padrões de conforto de pessoas submetidas a uma tecnologia de cuidar em Enfermagem por apresentarem pênfigo vulgar, uma doença cutâneo-mucosa rara que acomete principalmente adultos. A proposta aplicável em métodos experimentais com sujeitos submetidos à comparação quali-quantitativa (taxonomia/pertinência) do padrão de conforto antes e depois da intervenção. Requer o registro em escala cromática correspondente à intensidade de cada atributo: dor; mobilidade e comprometimento da autoimagem. As regras Fuzzy estabelecidas pela máquina de inferência definem o padrão de conforto em desconforto máximo, mediano e mínimo, traduzindo a eficácia dos cuidados de Enfermagem. Apesar de pouco utilizada na área de Enfermagem, essa lógica viabiliza pesquisas sem dimensionamento a priori do número de sujeitos em função da estimação de parâmetros populacionais. Espera-se avaliação do padrão de conforto do cliente com pênfigo diante da tecnologia aplicada de forma personalizada, conduzindo a avaliação global.

We present four cases of digestive bleeding whose skin manifestations guided diagnosis prior to endoscopy. These cases demonstrate the importance of a good physical examination of all patients rather than just focusing on laboratory tests [ABSTRACT FROM AUTHOR]

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.

El pénfigo es una enfermedad vesículo-ampollosa autoinmune de la piel y mucosas, que puede comprometer grandes extensiones; los tipos vulgar y foliáceo son los más frecuentes, con diferencias inmunopatológicas entre ellas. El pénfigo es tratado con corticoides y Azatioprina pero en los últimos años se incluyeron nuevos medicamentos como inmunoglobulinas, Rituximab (anticuerpo monoclonal quimérico anti-CD20) entre otros, Mostramos un caso de pénfigo hiperpigmentado atípico, y revisamos el tema en especial sobre el tratamiento. Salud UIS 2012; 44 (3): 49-55

The pemphigus foliaceus is a very common skin disease in the Center-West region of Brazil and is named endemic due to its incidence in some regions of the country, South America and Latin America. In Northeast Brazil, the pemphigus foliaceus is considered a rare disease. The bearers of endemic pemphigus foliaceus show an intense healing process associated with metabolic alterations that affect the skeletal muscle and can compromise in different ways the kinesiology of the locomotor apparatus. The aim of this study was to describe a pemphigus foliaceus case in Ceara state, focusing on the postural and functional aspects of the locomotor apparatus in the clinical evolution of the disease. Case report: A pediatric patient bearer of endemic pemphigus foliaceus, resident at a mountain region of Ceará, with clinical data consisting of erythematous lesions, crusts, few visible vesicle-blister lesions, intense itch, cutaneous adhesions, postural deformities and alterations in the flexor tonus of the limbs, compromising movement extent. The physiotherapeutic protocol prescribed consisted of active exercises, inhibition of flexor tonus, passive elongation and walk training. After 3 weeks, the patient was already strolling and showed improvement in muscular and joint function. The physiotherapy when precociously done can prevent the formation of cutaneous adhesions and muscular weakness, preserving the range of motion present in diseases with an intense healing process such as endemic pemphigus foliaceus.

Pênfigo Vegetante foi primeiramente descrito como uma variante do pênfigo vulgar, em 1876, por Neumann. Em 1889, Hallopeau descreveu um paciente com pústulas e placas vegetantes, e sugeriu ser uma variante do Pênfigo Vegetante de Neumann. Ambos os tipos de pênfigo vegetante são caracterizados pelo desenvolvimento de placas vegetantes, especialmente, em dobras (axila, inguinal, perianal). Os autores apresentam e discutem um caso de Pênfigo Vegetante com uma clínica incomum, com ausência de acometimento de mucosas e áreas de flexão, em paciente idosa, associado ao uso de enalapril como possível desencadeador. Diagnóstico clínico e histológico sugestivos de Pênfigo Vegetante tipo Hallopeau.Pemphigus Vegetans was first described as a variant of Pemphigus Vulgaris in 1876 by Neumann. In 1889, Hallopeau described a patient with pustules and vegetating plaques, suggesting that it would be a variant of Pemphigus Vegetans of Neumann. Both types of Pemphigus Vegetans are characterized by the development of vegetating plaques especially on skin folds (axillae, groin, perianal region). The authors present and discuss a case of Pemphigus Vegetans with an unusual clinical presentation lacking involvement of mucous membrane and flexor surfaces in an elderly female patient, associated with the use of enalapril as possible trigger factor. Clinical and histological diagnosis were suggestive of Pemphigus Vegetans of the Hallopeau type.