RESUMO
Immunofluorescence is a valuable auxiliary diagnostic tool for autoimmune bullous diseases and inflammatory disorders, since their clinical and histopathologic findings may be inconclusive. It is a feasible laboratory method that requires experienced technicians and detects in situ and circulating immune deposits that may be involved in the pathogenesis of such skin diseases.
Assuntos
Membrana Basal/química , Técnica Direta de Fluorescência para Anticorpo , Técnica Indireta de Fluorescência para Anticorpo , Dermatopatias/diagnóstico , Biópsia , Complemento C3/análise , Humanos , Imunoglobulinas/análise , Líquen Plano/diagnóstico , Líquen Plano/patologia , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Cutâneo/patologia , Pênfigo/diagnóstico , Pênfigo/patologia , Porfirias/diagnóstico , Porfirias/patologia , Dermatopatias/patologia , Vasculite/diagnóstico , Vasculite/patologiaRESUMO
A imunofluorescência é um valioso instrumento auxiliar no diagnóstico das dermatoses bolhosas autoimunes e desordens inflamatórias, uma vez que seus achados clínicos e histopatológicos podem não ser determinantes. Consiste em um método laboratorial factível, que requer profissionais técnicos experientes, e detecta imunocomplexos in situ e/ou circulantes, que podem estar envolvidos na patogênese de tais enfermidades cutâneas.
Immunofluorescence is a valuable auxiliary diagnostic tool for autoimmune bullous diseases and inflammatory disorders, since their clinical and histopathologic findings may be inconclusive. It is a feasible laboratory method that requires experienced technicians and detects in situ and circulating immune deposits that may be involved in the pathogenesis of such skin diseases.
Assuntos
Humanos , Membrana Basal/química , Técnica Direta de Fluorescência para Anticorpo , Técnica Indireta de Fluorescência para Anticorpo , Dermatopatias/diagnóstico , Biópsia , /análise , Imunoglobulinas/análise , Líquen Plano/diagnóstico , Líquen Plano/patologia , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Cutâneo/patologia , Pênfigo/diagnóstico , Pênfigo/patologia , Porfirias/diagnóstico , Porfirias/patologia , Dermatopatias/patologia , Vasculite/diagnóstico , Vasculite/patologiaRESUMO
Porphyrins are molecules essential for life. They are involved in the key processes of photosynthesis and respiration. The biosynthesis of tetrapyrroles in all living cells occurs through several steps where the formation of aminolevulinic acid (ALA) is the first committed intermediate. Two alternative routes for the formation of ALA have been proposed: one involves the condensation of Succinyl CoA and glycine catalyzed by ALA synthetase taking place in the mitochondria, and the second one is the so called 5-carbon route, occurring in the stroma of plastids. Eight molecules of ALA are used in the formation of protoporphyrin IX. Specific deficiencies in one of the enzymes of the heme pathway produce the porphyrias. In the acute porphyrias, the pathogenesis of the neurological dysfunction is attributed to the accumulation of ALA. Fluorescent and photosensitizing properties of protoporphyrin accumulated after the exogenous administration of ALA, can be used to visualize and destroy malignant cells in the so-called photodynamic diagnosis (PDD) and photodynamic therapy (PDT) of cancer. Many clinical ALA-PDT applications to malignant and non-malignant pathologies are currently in use. Different approaches to enhance ALA penetration in cells are under investigation, including the use of more lipophilic ALA derivatives and studies of the transport mechanisms of ALA. ALA has also been proposed to be used as a biodegradable herbicide, as an insecticide and as a plant growth regulator.
Assuntos
Ácido Aminolevulínico , Fotoquimioterapia , Fármacos Fotossensibilizantes , Animais , Diagnóstico por Imagem , Humanos , Neoplasias/diagnóstico por imagem , Neoplasias/tratamento farmacológico , Neoplasias/patologia , Oxigênio/metabolismo , Controle de Pragas , Fotossíntese/fisiologia , Porfirias/tratamento farmacológico , Porfirias/genética , Porfirias/patologia , Porfirinas/biossíntese , Radiografia , Tetrapirróis/biossínteseRESUMO
Hepatoerythropoietic porphyria (HEP) is the homozygous form of Porphyria Cutanea Tarda (PCT), characterized by an accumulation of porphyrins due to uroporphyrinogen decarboxylase deficiency. Fluorinated volatile anaesthetics are often used to produce general anaesthesia. Anaesthesia has certainly been implicated in the triggering of acute porphyria crisis. The effects of volatile anaesthetics in a B-lymphocyte cell line established from HEP patients (LBHEP) on heme metabolism have been investigated.LBHEP cells were exposed to sodium phosphate buffer containing dissolved Enflurane, Isoflurane or Sevoflurane (10mM) during 20min. Aminolevulinate synthase (ALA-S) activity, the regulatory enzyme of heme synthesis, was 300% induced by the anaesthetics. A 25-30% diminution of porphobilinogenase (PBG-ase) activity was found when Isoflurane or Sevoflurane were added to the cells, while no significant changes were detected after Enflurane treatment. Although some oxidative stress has been induced by the anaesthetics, reflected by the 35% diminution of glutathione (GSH), no alteration in heme oxygenase (HO) activity, the enzyme involved in heme breakdown and frequently induced as a response to stress stimuli, was observed. Studies using animals inoculated with LBHEP cells were also performed. Findings here described mimic biochemical alterations in the heme pathway, which are characteristic of another hepatic porphyria, similar to those previously reported when these anaesthetics were administered to animals, and they also advertise about the possible unsafe use of these drugs in the case of hepatic non-acute porphyrias.
Assuntos
Anestésicos Inalatórios/farmacologia , Anestésicos/farmacologia , Linfócitos B/efeitos dos fármacos , Porfirias/patologia , 5-Aminolevulinato Sintetase/farmacologia , Amônia-Liases/metabolismo , Animais , Enflurano/farmacologia , Heme/química , Humanos , Isoflurano/metabolismo , Isoflurano/farmacologia , Linfócitos/metabolismo , Éteres Metílicos/farmacologia , Camundongos , Camundongos Endogâmicos BALB C , Mutação , Estresse Oxidativo , Porfirias/tratamento farmacológico , Sevoflurano , Fatores de TempoRESUMO
The cutaneous deposition disorders are a group of unrelated conditions characterized by the presence of either endogenous or exogenous substances within the dermis or the subcutis. Part I of this two-part series will focus on metabolic processes involved in the endogenous deposition in the various forms of amyloidosis, porphyria, colloid milium, and lipoid proteinosis. We will also review the clinical, histologic, biochemical, and ultrastructural findings relevant to each disorder. Basic mechanisms of pathogenesis, diagnostic modalities, and treatment options are also discussed.
Assuntos
Humanos , Amiloidose/complicações , Amiloidose/diagnóstico , Amiloidose/etiologia , Amiloidose/metabolismo , Amiloidose/patologia , Amiloidose/terapia , Dermatopatias Metabólicas/diagnóstico , Dermatopatias Metabólicas/etiologia , Dermatopatias Metabólicas/metabolismo , Dermatopatias Metabólicas/patologia , Dermatopatias Metabólicas/terapia , Dermatopatias/diagnóstico , Dermatopatias/etiologia , Dermatopatias/metabolismo , Dermatopatias/patologia , Dermatopatias/terapia , Lipidoses/diagnóstico , Lipidoses/etiologia , Lipidoses/metabolismo , Lipidoses/patologia , Lipidoses/terapia , Pele/metabolismo , Pele/patologia , Porfirias/diagnóstico , Porfirias/etiologia , Porfirias/metabolismo , Porfirias/patologia , Porfirias/terapia , Proteinose Lipoide de Urbach e Wiethe/diagnóstico , Proteinose Lipoide de Urbach e Wiethe/etiologia , Proteinose Lipoide de Urbach e Wiethe/metabolismo , Proteinose Lipoide de Urbach e Wiethe/patologia , Proteinose Lipoide de Urbach e Wiethe/terapiaRESUMO
Se estudiaron 22 pacientes con PCT, 17 de ellos con biopsia hepática, presentando todos los casos alteraciones histológicas. Predominó la hepatitis crónica persistente. La deficiencia de UPG-asa en hígado tuvo relación directa con el daño hepático
Assuntos
Pessoa de Meia-Idade , Humanos , Masculino , Feminino , Fígado/fisiopatologia , Porfirias/fisiopatologia , Porfirinas/metabolismo , Fígado/patologia , Porfirias/patologiaRESUMO
Se estudiaron 22 pacientes con PCT, 17 de ellos con biopsia hepática, presentando todos los casos alteraciones histológicas. Predominó la hepatitis crónica persistente. La deficiencia de UPG-asa en hígado tuvo relación directa con el daño hepático (AU)
Assuntos
Pessoa de Meia-Idade , Humanos , Masculino , Feminino , Fígado/fisiopatologia , Porfirinas/metabolismo , Porfirias/fisiopatologia , Porfirias/patologia , Fígado/patologiaRESUMO
Se presenta un caso de Porfiria Cutánea Tarda en un pacientede 47 años , italiano,con antecedentes alcohólicos, visto en la consulta privadaquien presenta incremento de al fragilidad cutánea, formación de ampallas y vesículas a nivel del dorso de manos y antebrazos, de un año de evolución. La biopsia reportó: ampalla sub-epidermica.Sus orinas fueron de color rosadas. LOs niveles de uroporfirina en orina se encontraron bastante elevados. No presentó diábetes, pero si cifras moderadamente incrementadas de transaminasas