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Hereditary hemorrhagic telangiectasia

Synonym:

Osler-Rendu-Weber disease

Hereditary hemorrhagic telangiectasia are inherited as an autosomal dominant trait.
Punctate, somewhat domed, spider-like or linear telangiectasia are found anywhere on the body, but are especially common on the face. Lip involvement is typically a prominent feature, as is involvement of the nasal septum, leading to recurrent epistaxis.

Internal large vessel abnormalities can also be associated.

Gastrointestinal hemorrhage may occur.

Treatment:

  • Simple cautery is effective treatment for recurrent epistaxis.
  • The pulsed dye laser at 585 nm is the treatment of choice for cosmetically significant lesions.
telangiectasia hereditary


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