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.
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