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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

Upper endoscopy photos: Presence of multiple, friable, shiny telangiectasias inthe fornix and gastric body, and tiny angiectases in the second duodenalportion
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f03: Upper endoscopy photos: Presence of multiple, friable, shiny telangiectasias inthe fornix and gastric body, and tiny angiectases in the second duodenalportion

Mentions: Complementary exams revealed 8.5 g/dL hemoglobin, 79 fl. mean corpuscular volume (MCV),and 35µg/L serum ferritin, which is consistent with iron deficiency anemia. An upperendoscopy revealed angiectases (some were 3 mm in diameter, friable) in the fornix,gastric body and second duodenal portion (Figure3). CT angiography of the chest and computed cranial tomography showed noalterations.


Case for diagnosis. Hereditary Hemorrhagic Telangiectasia.

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

Upper endoscopy photos: Presence of multiple, friable, shiny telangiectasias inthe fornix and gastric body, and tiny angiectases in the second duodenalportion
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f03: Upper endoscopy photos: Presence of multiple, friable, shiny telangiectasias inthe fornix and gastric body, and tiny angiectases in the second duodenalportion
Mentions: Complementary exams revealed 8.5 g/dL hemoglobin, 79 fl. mean corpuscular volume (MCV),and 35µg/L serum ferritin, which is consistent with iron deficiency anemia. An upperendoscopy revealed angiectases (some were 3 mm in diameter, friable) in the fornix,gastric body and second duodenal portion (Figure3). CT angiography of the chest and computed cranial tomography showed noalterations.

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