ABSTRACT
RESUMO Objetivo: Relatar um caso de pênfigo neonatal em paciente que manifestou lesões cutâneas extensas e críticas ao nascimento. Descrição do caso: Recém-nascido do sexo masculino com lesões vesicobolhosas extensas em região anterior do tórax e abdome, desde o nascimento. Admitido na ala pediátrica de um hospital para diagnóstico etiológico e tratamento. Com base na história materna e na avaliação clínica, concluiu tratar-se de pênfigo vulgar neonatal. O paciente apresentou evolução satisfatória, sem a necessidade de intervenção farmacológica. Comentários: Os casos descritos na literatura e as referências avaliadas revelam o pênfigo neonatal como uma doença de ocorrência rara, porém cujo conhecimento e diagnóstico precoce têm grande relevância clínica, considerando-se que geralmente se manifesta com lesões epidérmicas extensas e de aspecto crítico, embora apresente curso clínico transitório e benigno, sem necessidade de tratamento específico e sem relação com doença futura.
ABSTRACT Objective: To report on the case of a patient with neonatal pemphigus that had extensive and critical skin lesions at birth. Case description: A newborn male with extensive vesico-bullous lesions on the anterior side of his chest and abdomen at birth. He was admitted to the pediatric ward of a hospital for an etiological diagnosis and for treatment. Based on maternal history and a clinical evaluation, the patient was diagnosed with neonatal vulgar pemphigus. His progression was satisfactory and, in the end, he did not need pharmacological interventions. Comments: The cases reported in the literature and the references evaluated reveal that neonatal pemphigus is rare, but that knowledge about the disease allows for an early diagnosis to be made. This has great clinical relevance considering that the disease usually manifests itself in the form of extensive epidermal lesions, even though it is transient and benign, it does not require specific treatment, and it does not have any relation with possible future diseases.
Subject(s)
Humans , Male , Female , Infant, Newborn , Adult , Infant, Newborn, Diseases/diagnosis , Remission, Spontaneous , Pemphigus/diagnosis , Pemphigus/physiopathology , Infant, Newborn, Diseases/physiopathology , Medical History Taking , MothersABSTRACT
Resumo Pênfigos são um grupo de doenças bolhosas autoimunes que acometem a pele e mucosas, cuja incidência mundial é de 0,75-5/1.000.000 casos ao ano. Nossa problematização parte das relações de poder/verdade e subjetivação que produzem modalidades de experiência sob a perspectiva hospitalar e das políticas públicas. Pesquisa qualitativa cartográfica, sustentada sob uma perspectiva Deleuziana. Nossos objetivos foram problematizar o pênfigo como doença-crônica e discutir as políticas de saúde e sua repercussão nas práticas de cuidado. Percorremos esta cartografia em um hospital filantrópico de referência nacional para tratamento de pênfigo. Esta pesquisa foi construída de setembro de 2012 a fevereiro de 2013. Os materiais de análise foram: prontuários, relatos em diário de campo de todas as atividades assistenciais realizadas, tais como: consultas médicas, procedimentos técnicos realizados pela equipe de enfermagem e equipe médica e relatos de pacientes, familiares, médicos, e equipe de enfermagem. Nossas análises foram construídas sob uma perspectiva histórico-genealógica Foucaultiana. Consideramos que as políticas de saúde a doentes crônicos com pênfigo não se apresentam como uma condição a ser evitada/prevenida, mas como uma anormalidade de grupos, bioidentidades que não têm efeito no conjunto da população.
Abstract Pemphigus is a group of autoimmune blistering diseases that affect the skin and mucous membranes, the global incidence of which is 0.75 to 5 / 1.000.000 cases a year. The approach taken derives from the relations of power/truth and subjectivity that produce modes of experience from the perspective of hospitals and public policies. It involves cartographic qualitative research, supported from a Deleuzian standpoint. The objective was to discuss pemphigus as a chronic disease and discuss health policies and their impact on care practices. This cartography was conducted in a philanthropical hospital, which is a national reference for the treatment of pemphigus. The research was conducted from September 2012 to February 2013. The analysis of materials included: medical records, field diary reports of all healthcare activities, such as medical consultations, technical procedures performed by nursing staff and medical staff and reports of patients, families, physicians, and nursing staff. The analyses were created from a Foucauldian historical and genealogical perspective. The conclusion reached is that health policies for chronic patients with pemphigus are not presented as a condition to be avoided / prevented, but as abnormal groups and bio identities that have no effect on the whole population.
Subject(s)
Humans , Public Policy , Pemphigus/therapy , Health Policy , Chronic Disease , Pemphigus/physiopathology , Qualitative ResearchABSTRACT
Abstract: Background: Pemphigus is part of a group of blistering diseases that affect the skin and mucous membranes. Based on its autoimmune origin, autoantibodies develop in pemphigus that are directed toward cell surface components of keratinocytes. However, some data cannot be explained, such as the lack of a relationship between autoantibody levels and the severity of clinical manifestations, treatment resistance, the presence of inflammatory infiltrates and the potential occurrence of apoptosis as determinants of vesicle formation. Objective: To examine the presence of apoptosis in pemphigus vulgaris by TUNEL technique. Methods: In this cross-sectional study, we selected 15 paraffin-embedded tissues from subjects who were diagnosed with pemphigus vulgaris by hematoxylin and eosin staining. The samples were subjected to TUNEL assay and examined under an Olympus BX61 fluorescence microscope. Positivity was categorized dichotomously, and the statistical analysis was performed using the X2 test. Results: Positivity was observed in basal layer cells in 14 (93.3%) cases. In 13 (86.7%) of the positive cases, we noted espinosum and granular layers that formed the blister roof, and in 12 cases (80%), positive acantholytic cells were observed. Conclusions: TUNEL positivity was observed in pemphigus vulgaris, implicating apoptosis in the pathophysiology of this condition, which can help guide the development of apoptotic blockers as therapeutics.
Subject(s)
Humans , Adult , Pemphigus/physiopathology , Apoptosis/physiology , In Situ Nick-End Labeling/methods , Skin/physiopathology , Cross-Sectional Studies , Acantholysis/physiopathology , Blister/physiopathology , Pemphigus/pathologyABSTRACT
Os autores relatam um caso de pênfigo vulgar de esôfago, destacando sua apresentação clínica inespecífica, o estabelecimento do diagnóstico através de endoscopia digestiva alta, histologia, imunofluorescência direta e a terapêutica clínica com glicocorticóides.
Subject(s)
Esophagus/physiopathology , Esophagus/pathology , Pemphigus/diagnosis , Pemphigus/physiopathology , Pemphigus/pathology , Pemphigus/therapy , EsophagoscopySubject(s)
Humans , Male , Female , Pemphigus/diagnosis , Pemphigus/physiopathology , Pemphigus/therapy , Diagnosis, DifferentialABSTRACT
EL pénfigo vulgar es una entidad autoinmune que puede comprometer piel y mucosas, exepcionalmente compromete el esófago. La presencia de anticuerpos antidesmosoma en la inmunofluorescencia es fundamental para el diagnóstico. En el diagnóstico diferencial se contemplan los pénfigoides, la necrólisis tóxica y la infección viral por herpes. El tratamiento requiere esteroides y citotóxicos que permitirían una mejor evolución del cuadro clínico. Informamos un caso de una mujer de 36 años con historia de pénfigo vulgar que ingresó por hematemesis, la endoscopia de vías digestivas altas mostró esfacelación de la mucosa compatible con el diagnóstico de pénfigo esofágico. Es tratada con esteroides revolviéndose el cuadro clínico y endoscópico
Subject(s)
Humans , Adult , Female , Pemphigus/diagnosis , Pemphigus/physiopathology , Pemphigus/complications , Pemphigus/therapyABSTRACT
Se presenta un caso de pénfigo vegetante de un año de evolución con respuesta muy satisfactoria al tratamiento combinado con prednisona y azatioprina durante 3 semanas