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Case for diagnosis. Hereditary Hemorrhagic Telangiectasia.

Boza JC, Dorn TV, Oliveira FB, Bakos RM - An Bras Dermatol (2014 Nov-Dec)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.

ABSTRACT
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.

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Related in: MedlinePlus

Telangiectasia on the oral mucosa. Lesions are also observed on the face
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f01: Telangiectasia on the oral mucosa. Lesions are also observed on the face

Mentions: A 73-year-old woman who was hospitalized for hematemesis had multiple, punctiformtelangiectasia lesions all over the integument since she was 40 years old. Lesions weremost prominent in the oral mucosa, on the tongue, hands and feet (Figures 1 and 2). She alsoreported recurrent episodes of nasal and intestinal bleeding. The patient underwenthysterectomy at age 39 due to menometrorrhagia. The son and grandson had similar skinlesions.


Case for diagnosis. Hereditary Hemorrhagic Telangiectasia.

Boza JC, Dorn TV, Oliveira FB, Bakos RM - An Bras Dermatol (2014 Nov-Dec)

Telangiectasia on the oral mucosa. Lesions are also observed on the face
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4230676&req=5

f01: Telangiectasia on the oral mucosa. Lesions are also observed on the face
Mentions: A 73-year-old woman who was hospitalized for hematemesis had multiple, punctiformtelangiectasia lesions all over the integument since she was 40 years old. Lesions weremost prominent in the oral mucosa, on the tongue, hands and feet (Figures 1 and 2). She alsoreported recurrent episodes of nasal and intestinal bleeding. The patient underwenthysterectomy at age 39 due to menometrorrhagia. The son and grandson had similar skinlesions.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.

ABSTRACT
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.

Show MeSH
Related in: MedlinePlus