Syndromes that predispose to epistaxis.

Purpose: Our aim was to evaluate if epistaxis is directly associated with the etiology or pathophysiological mechanism which results in the syndrome itself or arises as a secondary effect.
Methods: We performed an extensive literature review of the web-based PubMed database from the National Library of Medicine to ascertain syndromes related to this condition. Etiology, pathophysiological mechanisms, occurrence, clinical features and management were noted for each of these syndromes.
Results: Epistaxis is commonly seen in syndromes that are usually directly related to vascular abnormalities or coagulation defects. However, in some cases, it is not.
Discussion and Conclusion: Since a number of these syndromes are rare and elaborate tests are not carried out in the absence of a positive family history or until other specific clinical features appear, a risk of underdiagnosis and the dilemma of whether epistaxis is specifically related to the syndrome or a secondary effect still remains.