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Otolaryngol Clin North Am. 2018 Feb;51(1):237-254. doi: 10.1016/j.otc.2017.09.017.

Hereditary Hemorrhagic Telangiectasia.

Author information

1
University of Regensburg, Franz-Josef-Strauss-Allee 11, D-93053 Regensburg, Germany. Electronic address: Thomas.Kuehnel@ukr.de.
2
University of Regensburg, Franz-Josef-Strauss-Allee 11, D-93053 Regensburg, Germany.

Abstract

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.

KEYWORDS:

Dysplasias; Hereditary hemorrhagic telangiectasia; Mucocutaneous lesions; Osler-Weber-Rendu syndrome

PMID:
29217066
DOI:
10.1016/j.otc.2017.09.017
[Indexed for MEDLINE]

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