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URTICÁRIA Da Clínica À Terapêutica

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URTICÁRIA Da Clínica À Terapêutica URTICÁRIA da Clínica à Terapêutica URTICÁRIA da Clínica à Terapêutica editor Celso Pereira © Autor Merck Sharpe & Dohme (MSD) Título: Urticária da Clínica à Terapêutica Editor: Celso Pereira Imagem da capa: Arq Pedro Rodrigues (Coimbra) Design e Produção Gráfica: Nastintas – Design e Comunicação (Lisboa) Impressão: Offsetmais Artes Gráficas, S.A. ISBN: 978-989-96725-0-5 Depósito Legal: XXXXX Tiragem: 8000 exemplares Data de edição: Maio 2010 Reservados todos os direitos. Sem prévio consentimento da editora, não poderá reproduzir-se, nem armazenar-se num suporte recuperável ou transmissível, nenhuma parte desta publicação, seja de forma electrónica, mecânica, fotocopiada, gravada ou por qualquer outro método. Todos os comentários e opiniões publicados neste livro são de responsabilidade exclusiva dos seus autores. “Nesta nossa profissão, como muito bem sabem quantos a exercem, podem acontecer milagres e até se diz que a Medicina tem muito de Divino, mas temos que estar sempre atentos a todos os pormenores e aos mais pequenos sinais.” In Curationum Medicinalium Centuriae Septem Amatus Lusitanus (1511-1568) VII NOTA INTRODUTÓRIA O uso eficiente e disciplinado da informação e do conhe- cimento científico são valores essenciais para a evolução da prática da Medicina. A aplicação eficiente de conhecimentos médicos actua- lizados exige uma gestão criteriosa da informação que é disponibilizada e divulgada diariamente Neste contexto, é indiscutível a necessidade e a pertinên- cia da disponibilização de guias de orientação e padrões de actuação, que se traduzem no contexto das boas práticas em Normas Orientadoras de Diagnóstico e Tratamento. Apesar de não se tratar de uma doença de elevada prevalência, a Urticária tem um impacto significativo na qualidade de vida dos doentes, constituindo um desafio pela sua diversidade etiológica, formas de apresentação e consequente complexidade de diagnóstico e abordagem terapêutica. É por isso de relevar, a iniciativa dos autores que, conscientes destes factos, reconheceram a necessidade da implementação de uma abordagem multidisciplinar, racional e orientada para o diagnóstico e tratamento da Urticária. Para além de incluir as mais recentes actualizações cien- tíficas nesta área, a estrutura desta publicação permite a sua utilização como referência na abordagem individuali- zada a cada doente. Reconhecendo o inequívoco valor deste tipo de inicia- tivas na formação médica contínua, quero em nome da MSD congratular os autores e deixar expresso o nosso agradecimento pela oportunidade de pareceria na cria- ção de divulgação desta obra de valor inquestionável. Ana Maria Nogueira Directora Médica MSD IX PREFÁCIO O avanço no conhecimento médico em relação à urticá- ria substancia e contextualiza a edição de “Urticária, da Clínica à Terapêutica”. Esta edição sucede a uma outra, também editada pelo Doutor Celso Pereira, em 2001, “Urticária. Imunopatologia, clínica e terapêutica”. No período que medeia as duas edições, a evolução de conceitos nos domínios da biologia molecular e da gené- tica tiveram uma repercussão enorme no conhecimento da fenomenologia da resposta imuno-inflamatória com sede, ou repercussão, cutânea. Este salto, notável, justifica plenamente a actualização e alargamento dos conteúdos da primeira publicação. A este propósito deve saudar-se o capítulo inicial da presente edição, dedicado ao “Sistema Imune Cutâneo” e escrito pelo insigne Professor Antero da Palma Carlos. A pele é uma extensa interface que evoluiu com a espécie no sentido de se defender do meio externo. Mas, a visão da pele ser apenas uma barreira em relação à agressão externa – epitélio estratificado, camada córnea e a sua riqueza em lipídos – é reducionista, já que tam- bém a preservação do meio interno, caso da retenção de água, é, como se compreende, um aspecto fisiológico essencial. Acresce a existência de meios de defesa inata, como a presença de péptidos antimicrobianos, e a distri- buição estratégica de células diferenciadas para o reco- nhecimentos de padrões moleculares microbianos, para a informação antigénica e desenvolvimento de imunidade adquirida, para a regulação da resposta inflamatória. Por outro lado, a extensa rede capilar cutânea confere à pele um potencial enorme de ocorrências imuno- inflamatórias locais, mais ou menos extensas, quando se verificam respostas anómalas e desequilíbrios imunitários, de que é paradigma a patologia do conectivo (vasculite urticariana, urticária autoimune,…). X Na esfera da urticária ainda ocorrem alguns equívocos na prática médica, para não falar dos que se constatam antes da observação médica, em consulta ou no servi- ço de urgência. A perspectiva de, face a uma urticária, estarmos perante uma síndrome, ou apenas um sintoma, é válida e útil para se compreender o carácter multifac- torial da patologia e para incitar ao estudo detalhado do enquadramento clínico. Até neste particular o pre- sente texto é útil; ao situar, com manifesta clareza, as diversas formas de génese e expressão de urticária, e ao procurar orientar o seu estudo de forma criteriosa e selectivamente dirigido. A incidência cumulativa de urticária aguda nos países do ocidente é considerável. Com efeito, 20 a 25% da popu- lação regista a ocorrência de pelo menos um episódio de urticária aguda. A urticária crónica – por definição, com pelo menos 6 semanas de duração –, tem uma pre- valência relativamente reduzida; todavia, esta forma de urticária reune alguma complexidade para um adequado enquadramento clínico. É oportuno referir que o atribu- to de idiopática, com que frequentemente é conotada, deverá, no entanto, ser muito menos utilizado. Também aqui, neste livro, é explicado porquê. A urticária física, caracterizada pelo seu polimorfismo clínico de acordo com o agente causal, é extensivamente tratada num texto de muito boa qualidade. Não menos relevante é a participação de Colegas dermatologistas nos capítulos de urticária de contacto e de hidratação cutânea. A repercussão da urticária crónica na qualidade de vida e os aspectos psicológicos relacionados com o tema são também uma mais valia numa obra com este cariz. Cabe-me, pois, felicitar vivamente o editor, Doutor Celso Pereira, e todos os autores que contribuíram para a ex- celente qualidade desta obra. António Segorbe Luís Professor Associado da Faculdade de Medicina da Universidade de Coimbra. Regente de Imunologia Clínica. XI AUTORES AG Palma Carlos Prof. Catedrático Jubilado de Medicina Interna, Imunologia e Imunoalergologia da Faculdade de Medicina, Universidade de Lisboa Anabela Lopes Assistente de Imunoalergologia, Hospital Santa Maria, Centro Hospitalar Lisboa Norte. EPE Beatriz Tavares Assistente Imunoalergologia, Hospitais da Universidade de Coimbra, EPE Carlos Fernandes da Silva Professor Catedrático de Psicologia da Universidade de Aveiro Cátia Barbosa Licenciada em Ciências Farmacêuticas Celso Pereira Assistente Graduado Imunoalergologia, Hospitais da Universidade de Coimbra, EPE Doutorado em Medicina Interna; Patologia Médica, Medicina Dentária, Faculdade Medicina, Universidade Coimbra Cristina Santa Marta Especialista Imunoalergologia, Hospital CUF/Descobertas, Lisboa Daniel Machado Interno de Imunoalergologia, Hospitais da Universidade de Coimbra, EPE Fernanda Ferrão Assistente Graduada de Anestesiologia, Hospital Arcebispo João Crisóstomo, Cantanhede ; Licenciada em Psicologia Graça Loureiro Assistente Imunoalergologia, Hospitais da Universidade de Coimbra, EPE José Carlos Cardoso Assistente Dermatologia, Hospitais da Universidade de Coimbra, EPE XII M Graça Castel-Branco Directora Serviço Imunoalergologia, Hospital São João EPE, Porto Manuel Branco-Ferreira Assistente de Imunoalergologia, Hospital Santa Maria, Centro Hospitalar Lisboa Norte. EPE Assistente de Imunologia Clínica e Medicina Interna, Faculdade Medicina, Universidade de Lisboa Margarida Gonçalo Chefe Serviço Dermatologia, Hospitais da Universidade de Coimbra, EPE Margarida Robalo Cordeiro Assistente Dermatologia, Hospitais da Universidade de Coimbra, EPE Mário Morais Almeida Coordenador do Serviço Imunoalergologia, Hospital CUF/Descobertas, Lisboa Sociedade Portuguesa de Alergologia e Imunologia Clínica Paula Leiria Pinto Coordenadora do Serviço Imunoalergologia, Hospital Dona Estefânea, Centro Hospitalar Lisboa Central, EPE. Victor Guerra Licenciado em Psicologia XIII SUMÁRIO 1 Introdução: Celso Pereira 17 2 Órgão imune cutâneo 23 Sistema imunitário cutâneo: AG Palma Carlos Inflamação cutânea: Manuel Branco Ferreira 3 Clínica e classificação da urticária: Celso Pereira 51 4 Urticária aguda: Cristina Santa Marta 69 5 Urticária crónica 91 Recorrente: Celso Pereira Formas particulares Autoimune: Beatriz Tavares Associação a doença sistémica: Graça Loureiro 6 Urticária física: Mário Morais Almeida 191 7 Urticária contacto: Margarida Gonçalo 223 8 Urticária, formas especiais: Anabela Lopes 255 9 Particularidades na criança: Paula Leiria Pinto 275 10 Hidratação cutânea: Margarida Robalo Cordeiro, 283 José Carlos Cardoso 11 Implicações da urticária na qualidade de vida: 299 M Graça Castel-Branco 12 Aspectos psicológicos no doente com urticária 309 Perfil do doente: Fernanda Ferrão, Vítor Guerra, Carlos Fernandes da Silva Estratégias de apoio: Carlos Fernandes da Silva, Fernanda Ferrão, Vítor Guerra Adenda Alergénios/Condicionantes ocultos: 337 Daniel Machado, Cátia Barbosa XV ÍNDICE GERAL 1 Introdução 19 2 Órgão imune cutâneo 25 – Sistema imunitário cutâneo 27 Histologia e fisiologia da pele 27 A epiderme, barreira cutânea 27 Péptidos epiteliais antimicrobianos 28 Células
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