Palmoplantar keratoderma-esophageal carcinoma syndrome

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Palmoplantar keratoderma-esophageal carcinoma syndrome

Synonyms: Bennion-Patterson syndrome | Howell-Evans syndrome | Keratosis palmoplantaris-esophageal carcinoma syndrome | Palmoplantar hyperkeratosis-esophageal carcinoma syndrome | Tylosis-oesophageal carcinoma syndrome

A rare genetic disease characterized by thickening of the skin on palms and soles restricted to areas of weight bearing and/or friction (focal non-epidermolytic palmoplantar keratoderma) and oral and esophageal leukokeratosis associated with a very high lifetime risk of developing squamous cell carcinoma of the esophagus. The skin lesions appear in childhood and can be complicated by fissuring and infection.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: // Data version September 2023.

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Advocacy Organizations

My Faulty Gene

My Faulty Gene is a nonprofit organization which provides information and assistance to any individual whose family medical history suggests genetic testing might be helpful in identifying an increased risk of disease due to a genetic mutation. We believe that everyone in need of genetic testing should have access to it.

My Little Sunshine Foundation

My Little Sunshine is a non-profit foundation dedicated to educating people about the importance of fertility preservation and making fertility resources accessible to all.


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Clinical Trials

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