Rendu-Osler-Weber syndrome: dermatological approach

被引:2
作者
Barbosa, Aline Blanco [1 ]
Hans Filho, Guenter [2 ]
dos Santos Vicari, Carolina Faria [1 ]
Medeiros, Marcelo Zanolli [2 ]
Couto, Daine Vargas [2 ]
Takita, Luiz Carlos [2 ]
机构
[1] Hosp Sao Juliao, Campo Grande, MS, Brazil
[2] Univ Fed Mato Grosso Sul UFMS, Campo Grande, MS, Brazil
关键词
Epistaxis; Genetics; Telangiectasia; hereditary hemorrhagic; HEREDITARY HEMORRHAGIC TELANGIECTASIA;
D O I
10.1590/abd1806-4841.20152563
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
The Rendu-Osler-Weber syndrome is a rare systemic fibrovascular dysplasia, recognized by mucocutaneous telangiectasias, arteriovenous malformations, epistaxis and family history. Recurrent bleeding, hypoxemia, congestive heart failure, portosystemic encephalopathy, and symptoms related to angiodysplasia of the central nervous system may occur. Since the treatment is based on supportive measures, early recognition is of utmost importance. This article reports the case of a 53-year-old male patient who presented telangiectasias on fingers, oral cavity and nasal mucosa for 10 years, with a history of recurrent epistaxis of varying severity since childhood. Mother, sister and daughter have similar lesions.
引用
收藏
页码:226 / 228
页数:3
相关论文
共 7 条
[1]   Bevacizumab in Hereditary Hemorrhagic Telangiectasia [J].
Bose, Prithviraj ;
Holter, Jennifer L. ;
Selby, George B. .
NEW ENGLAND JOURNAL OF MEDICINE, 2009, 360 (20) :2143-2144
[2]   Hereditary hemorrhagic telangiectasia: from molecular biology to patient care [J].
Dupuis-Girod, S. ;
Bailly, S. ;
Plauchu, H. .
JOURNAL OF THROMBOSIS AND HAEMOSTASIS, 2010, 8 (07) :1447-1456
[3]   Hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease) [J].
Fuchizaki, U ;
Miyamori, H ;
Kitagawa, S ;
Kaneko, S ;
Kobayashi, K .
LANCET, 2003, 362 (9394) :1490-1494
[4]  
Garcia Roberta I. D., 2003, Rev. Bras. Otorrinolaringol., V69, P577, DOI 10.1590/S0034-72992003000400022
[5]  
Gontijo Bernardo, 2004, An. Bras. Dermatol., V79, P7
[6]  
Juares Antônio José Cortez, 2008, Rev. Bras. Otorrinolaringol., V74, P452, DOI 10.1590/S0034-72992008000300022
[7]   Hereditary hemorrhagic telangiectasia:: an update on clinical manifestations and diagnostic measures [J].
Sadick, H ;
Sadick, M ;
Götte, K ;
Naim, R ;
Riedel, F ;
Bran, G ;
Hörmann, K .
WIENER KLINISCHE WOCHENSCHRIFT, 2006, 118 (3-4) :72-80