Paper
Tylosis associated with carcinoma of the oesophagus and oral leukoplakia in a large Liverpool family—A review of six generations

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      Esophageal lichen planus is associated with the development of ESCC in up to 6.1% of patients.24 Tylosis or hyperkeratosis palmaris et plantaris is a rare but autosomal dominant genetic disease marked by aberrations of the RHBDF2 gene resulting in a significant risk of ESCC of at least 80% in a small kindred.25,26 Human papillomavirus has also been associated with the development of ESCC.27

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      Only late onset tylosis (type A), which typically presents between 5 and 15 years old, is associated with increased ESCC risk [39,40]. Lifetime risk of developing ESCC is very high, estimated from 40% to 95% [39,41]. Results of endoscopic surveillance program of one of the tylosis families showed that 5 out of 6 cases of ESCC were detected endoscopically and were surgically removed.

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