RESUMEN
Cicatricial pemphygoid (mucous membrane cicatricial pemphygoid) is a chronic autoimmune inflammatory disease characterized by subepithelial bubbles in mucous membranes and, occasionally on the skin. It may affect the mouth, the nose, pharynx, larynx, the eyes, esophagus, anus, genitals and skin; especially affecting patients between fifty and sixty years of life. Treatment includes systemic steroids and immunosuppressive agents. In the present paper we describe two cases with the active disease, and one of them had sepsis because of using immunosuppressive agents and another that presented supraglottic stenosis requiring tracheostomy.
Asunto(s)
Enfermedades de la Boca/etiología , Penfigoide Benigno de la Membrana Mucosa/complicaciones , Anciano , Humanos , Masculino , Persona de Mediana Edad , Enfermedades de la Boca/diagnóstico , Enfermedades de la Boca/tratamiento farmacológico , Penfigoide Benigno de la Membrana Mucosa/diagnóstico , Penfigoide Benigno de la Membrana Mucosa/tratamiento farmacológicoRESUMEN
Fanconi's anemia is a rare autosomal recessive disorder characterized by congenital malformation, bone marrow failure and genomic instability, with a predisposition to develop malignancies, especially the leukemias and upper aerodigestive tract tumors. Due to inherent characteristics to this syndrome, the treatment of such neoplasms is particularly difficult. In this paper we report the case of a 24-year-old woman with Fanconi's anemia who developed a squamous cell carcinoma of the hypopharynx; she had none of the traditional risk factors, such as smoking and alcohol abuse. We also briefly review the literature about this topic.
Asunto(s)
Carcinoma de Células Escamosas/complicaciones , Anemia de Fanconi/complicaciones , Neoplasias Faríngeas/complicaciones , Adulto , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/radioterapia , Resultado Fatal , Femenino , Humanos , Hipofaringe , Neoplasias Faríngeas/diagnóstico , Neoplasias Faríngeas/radioterapiaRESUMEN
Penfigóide Cicatricial (Penfigóide Cicatricial de Membrana Mucosa) é uma doença auto-imune inflamatória crônica caracterizada pela presença de bolhas subepiteliais em membranas mucosas e, ocasionalmente na pele. Pode haver acometimento oral, nasal, faríngeo, laríngeo, ocular, esofágico, anogenital e de pele, predominantemente em pacientes na quinta e sexta décadas de vida. O tratamento preconizado inclui corticóides sistêmicos e agentes imunossupressores. Descrevemos neste trabalho dois casos com a doença em atividade, sendo que um evoluiu com complicação séptica pelo uso de imunossupressor e outro que apresentou estenose supraglótica exigindo traqueotomia.
Cicatricial pemphygoid (mucous membrane cicatricial pemphygoid) is a chronic autoimmune inflammatory disease characterized by subepithelial bubbles in mucous membranes and, occasionally on the skin. It may affect the mouth, the nose, pharynx, larynx, the eyes, esophagus, anus, genitals and skin; especially affecting patients between fifty and sixty years of life. Treatment includes systemic steroids and immunosuppressive agents. In the present paper we describe two cases with the active disease, and one of them had sepsis because of using immunosuppressive agents and another that presented supraglottic stenosis requiring tracheostomy.
Asunto(s)
Anciano , Humanos , Masculino , Persona de Mediana Edad , Enfermedades de la Boca/etiología , Penfigoide Benigno de la Membrana Mucosa/complicaciones , Enfermedades de la Boca/diagnóstico , Enfermedades de la Boca/tratamiento farmacológico , Penfigoide Benigno de la Membrana Mucosa/diagnóstico , Penfigoide Benigno de la Membrana Mucosa/tratamiento farmacológicoRESUMEN
A anemia de Fanconi é um raro distúrbio autossômico recessivo caracterizado por malformações congênitas, aplasia da medula óssea e instabilidade genômica, com predisposição ao desenvolvimento de neoplasias malignas, em especial as leucemias e os tumores do trato aerodigestivo alto. Em razão de características inerentes à síndrome em questão, o tratamento de tais neoplasias é particularmente difícil. Relata-se o caso de anemia de Fanconi uma jovem de 24 anos, que desenvolveu carcinoma de células escamosas da hipofaringe, na ausência de fatores de risco como o tabagismo e o alcoolismo, e faz-se uma revisão sumária da literatura a respeito do tema.
Fanconi's anemia is a rare autosomal recessive disorder characterized by congenital malformation, bone marrow failure and genomic instability, with a predisposition to develop malignancies, especially the leukemias and upper aerodigestive tract tumors. Due to inherent characteristics to this syndrome, the treatment of such neoplasms is particularly difficult. In this paper we report the case of a 24-year-old woman with FanconiÆs anemia who developed a squamous cell carcinoma of the hypopharynx; she had none of the traditional risk factors, such as smoking and alcohol abuse. We also briefly review the literature about this topic