Case for diagnosis. Hereditary Hemorrhagic Telangiectasia.
The Osler-Weber-Rendu syndrome or Hereditary Hemorrhagic Telangiectasia (HHT) is a systemic fibrovascular dysplasia characterized by defects in the elastic and vascular walls of blood vessels, making them varicose and prone to disruptions. Lesions occur in different organs and can lead to hemorrhage in the lungs, digestive tract and brain. We describe the case of a patient with cutaneous manifestations and severe impairment of the digestive tract. It is important for the dermatologist to recognize this syndrome, since the cutaneous lesions may play a key role in diagnosis.
Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
Juliana Catucci
JC
Boza
Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
Timotio Volnei
TV
Dorn
Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
Fabiana Bazanella de
FB
Oliveira
Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
Renato Marchiori
RM
Bakos
D000368:Aged; D005260:Female; D005773:Gastroscopy; D006801:Humans; D009061:Mouth Mucosa / Q000473:pathology; D012867:Skin / Q000473:pathology*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000473:pathology*; D014059:Tongue / Q000473:pathology