Mucocutaneous telangiectases of the head and neck in individuals with hereditary hemorrhagic telangiectasia -- analysis of distribution and symptoms.
Telangiectases are a diagnostic clue of hereditary hemorrhagic telangiectasia (HHT, Rendu-Osler-Weber disease), but they are not specific to HHT. The characteristic features of telangiectases were studied in a group of 70 individuals with this disorder. The files, including photo and video documentation of these patients, were reviewed with regard to mucocutaneous vascular lesions. Telangiectases could be identified within the nasal mucosa in 90% of the HHT patients. Extranasal telangiectases were identified in descending order in the oral cavity, the facial skin, the hands, the auricles and the thorax. The vascular lesions showed considerable variation in size and shape, and on the nasal mucosa they were most commonly hemorrhagic. The earliest onset of cutaneous telangiectases was documented at the age of 6 years. Contrary to previous communications, more than 25% of patients had hemorrhages outside the nose. These hemorrhages were self-limiting in most cases. Prolonged hemorrhages requiring treatment were observed in 12% of cases. Such severe hemorrhages originated from telangiectases of the base of the tongue, the body of the tongue, the fingers and the skin of the supraclavicular fossa. We conclude that telangiectases occur at an earlier age than generally thought and are a hallmark of a serious disorder rather than a cosmetic problem.
Folz
Benedikt J
BJ
Department of Otolaryngology, Head and Neck Surgery, Philipp University of Marburg, Deutschhausstr. 3, 35037 Marburg, Germany. folz@med.uni-marburg.de
Lippert
Burkard M
BM
Wollstein
Ana Cerra
AC
Tennie
Julia
J
Happle
Rudolf
R
Werner
Jochen A
JA
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D005260:Female; D020022:Genetic Predisposition to Disease; D005858:Germany / Q000453:epidemiology; D006257:Head / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009333:Neck / Q000473:pathology; D012720:Severity of Illness Index; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology