Hereditary Hemorrhagic Telangiectasia

Publication date: February 2018
Source:Otolaryngologic Clinics of North America, Volume 51, Issue 1
Author(s): Thomas Kühnel, Kornelia Wirsching, Walter Wohlgemuth, Ajay Chavan, Katja Evert, Veronika Vielsmeier

Teaser

Hereditary hemorrhagic telangiectasia (HHT) describes the presenting manifestations of a disorder that is characterized by pathologic blood vessels. HHT is inherited as an autosomal dominant trait with variable penetrance. The abnormal vascular structures (dysplasias) can affect all the organs in the human body. The link between a physical stimulus and new lesion development has been established for mucosal trauma owing to nasal airflow turbulence, for ultraviolet exposure to the fingers, and for mechanical trauma to the dominant hand. The pressing question then is whether HHT treatment constitutes a stimulus that is sufficient to trigger new lesion development.


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