Original scientific paper

The Importance of a Multidisciplinary Approach in a Patient with Long-term Multisystemic Manifestations of Unrecognized Hereditary Hemorrhagic Telangiectasia

Vesna Sredoja Tišma ; Dermatovenerology Unit, Dubrava University Hospital, Zagreb, Croatia
Ana Vlašić ; Department of Otorhinolaryngology and Head & Neck Surgery, Dubrava University Hospital, Zagreb, Croatia
Petar Gulin ; Department of Otorhinolaryngology and Head & Neck Surgery, Dubrava University Hospital, Zagreb, Croatia
Renata Huzjan Korunić ; Clinical Department of Diagnostic and Interventional Radiology, Dubrava University Hospital, Zagreb, Croatia

Abstract

ABSTRACT Hereditary hemorrhagic telangiectasia (HHT), also called Rendu-Osler-Weber syndrome, is a rare autosomal dominant multisystemic vascular disorder, characterized by widespread mucocutaneous teleangiectasias, frequent visceral arteriovenous malformations (AVM) and a tendency for bleeding. This diagnosis should be suspected in all dermatological patients with generalized mucocutane-ous vascular lesions at sites of predilection, associated frequent epistaxis and a positive family history. The aim of this paper is to emphasize the importance of a multidisciplinary approach, the role and timely cooperation of dermatologists and otorhinolaryngologists in the early clinical recognition and diagnosis of the disease. We present a family case of a 63-year - old patient with typical clinical features of HHT and long-standing multisystemic complications of unrecognized disease.

Keywords

hereditary hemorrhagic teleangiectasia; multidisciplinary approach; mul-tisystemic manifestations

Hrčak ID:

293997

URI

https://hrcak.srce.hr/293997

Publication date:

2.5.2022.

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