Revista de Odontologia da UNESP
https://revodontolunesp.com.br/article/588018ce7f8c9d0a098b4e20
Revista de Odontologia da UNESP
Original Article

Hereditary hemorrhagic telangiectasia: case report

Telangiectasia hemorrágica hereditária: relato de caso

Sampieri, Marcelo Bonifácio da Silva; Oliveira-Santos, Christiano de; Duarte, Bruno Gomes; Capelozza, Ana Lúcia Álvares; Damante, José Humberto

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Abstract

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder characterized by an aberrant vascular development and by the presence of multiples mucocutaneous telangiectases, commonly affecting lips, tongue, feet, hands and nasal cavity. Besides the disease can be responsible for the occurrence of nosebleed. We report a case of a 54-year-old female patient that was refered to our clinic with the complaint of an erythematous lesion in the lower lip. Clinical examination showed us multiple erythematous papules in oral mucosa, skin, face and hands. Fhurtermore she had a history of epistaxis and familial occurence, confirming HHT. Due to absence of symptoms, follow up of the lesion was advised. The objective of this paper is report a case of Hereditary hemorrhagic telangiectasia as well as the types of treatment and complications inherent to this disease.

Keywords

Hereditary hemorrhagic telangiectasia, epistaxis, Osler Rendu disease

Resumo

A telangiectasia hemorrágica hereditária (THH) apresenta-se como um distúrbio autossômico dominante, caracterizado pelo desenvolvimento vascular aberrante e pela presença de múltiplas telangiectasias mucocutâneas, acometendo principalmente lábios, língua, pele dos pés e das mãos. Pode ainda acometer a mucosa nasal, sendo então responsável pela ocorrência de epistaxes nasais. No presente caso, paciente de 54 anos de idade, do gênero feminino, compareceu à Clínica de Estomatologia da Faculdade de Odontologia de Bauru, queixando-se de uma lesão avermelhada no lábio inferior. Após o exame físico e a anamnese, observou-se a presença de múltiplas pápulas avermelhadas na mucosa oral, na pele, na face e nas mãos, bem como história de epistaxe e acometimento familiar, confirmando a THH. Em virtude da ausência de sintomatologia, optou-se pela proservação da paciente. O objetivo deste artigo é relatar um caso de telangiectasia hemorrágica hereditária, bem como os tipos de tratamento e complicações inerentes à doença.

Palavras-chave

Telangiectasia hemorrágica hereditária, epistaxe, doença de Osler Rendu

References



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