Hereditary hemorrhagic telangiectasia is a rare disease, probably due to mesenchymal dysplasia. It is characterized by the presence of multiple acquired angiomas or telangiectases of varying distribution and number with a marked tendency to bleed spontaneously or from slight trauma. The disease is not sex bound. It is transmitted as a dominant characteristic. The initial symptom usually consists of abnormally profuse epistaxis beginning about puberty. This is followed by the development of multiple telangiectasia of the skin and mucous membranes from the age of 25 to 35. Hemorrhage from the nasal mucosa is most frequent, and all symptoms tend to reach their greatest severity during the fourth decade. The average mortality rate is 4 per cent.
The lesions are most commonly found in the nasal mucosa, the skin of the face, the buccal mucosa, the lips, the tongue, the floor of the mouth, the scalp, the ears, the conjunctivas and