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Síndrome de Melkersson-Rosenthal : um diagnóstico a ponderar numa doente com edema do lábio

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Detalhes bibliográficos
Resumo:Melkersson-Rosenthal Syndrome is a rare neuromucocutaneous disease with a prevalence of 0.08% worldwide. This syndrome is characterized by the triad of recurrent orofacial edema, fissured tongue and facial palsy. The most common clinical feature is the recurrent edema, which is characteristically acute, non-tender and confined to the lips. When isolated, the recurrent edema of the lip is called Granulomatous Cheilitis or Meisher’s Syndrome. The case described refers to a 43 year old patient with recurrent edema of the upper lip and a positive biopsy for granulomatous cheilitis. The patient was initially treated with corticosteroids, with poor response. She then started intralesion injections, with symptomatic improvement. The treatment is still controversial, as the etiology remains unknown. In this article the diagnostic approach, the histophatoly and the treatment of this rare syndrome are discussed.
Autores principais:Teixeira, Raquel Filipa Corda
Assunto:Síndrome de Melkersson-Rosenthal
Ano:2014
País:Portugal
Tipo de documento:dissertação de mestrado
Tipo de acesso:acesso restrito
Instituição associada:Universidade de Lisboa
Idioma:português
Origem:Repositório da Universidade de Lisboa
Descrição
Resumo:Melkersson-Rosenthal Syndrome is a rare neuromucocutaneous disease with a prevalence of 0.08% worldwide. This syndrome is characterized by the triad of recurrent orofacial edema, fissured tongue and facial palsy. The most common clinical feature is the recurrent edema, which is characteristically acute, non-tender and confined to the lips. When isolated, the recurrent edema of the lip is called Granulomatous Cheilitis or Meisher’s Syndrome. The case described refers to a 43 year old patient with recurrent edema of the upper lip and a positive biopsy for granulomatous cheilitis. The patient was initially treated with corticosteroids, with poor response. She then started intralesion injections, with symptomatic improvement. The treatment is still controversial, as the etiology remains unknown. In this article the diagnostic approach, the histophatoly and the treatment of this rare syndrome are discussed.