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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Tylosis with esophageal cancer


Other Names for this Disease

  • Howel-Evans syndrome
  • Keratosis palmaris et plantaris with esophageal cancer
  • Keratosis palmoplantaris with esophageal cancer
  • TOC
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Symptoms

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What are the signs and symptoms of Tylosis with esophageal cancer?

The main features of Tylosis with esophageal cancer are palmoplantar keratoderma and esophageal cancer. The palmoplantar keratoderma usually begins around age 10, and the soles of the feet are usually more severely affected that the palms of the hands. Esophageal carcinoma usually develops in the lower two-thirds of the esophagus at an average age of 45 years.[1]
Last updated: 1/18/2013

The Human Phenotype Ontology provides the following list of signs and symptoms for Tylosis with esophageal cancer. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Abnormality of the intestine 90%
Esophageal neoplasm 90%
Gastrointestinal hemorrhage 90%
Nausea and vomiting 90%
Palmoplantar keratoderma 90%
Ascites 50%
Feeding difficulties in infancy 50%
Hepatomegaly 50%
Mediastinal lymphadenopathy 50%
Weight loss 50%
Clubbing of toes 7.5%
Vocal cord paresis 7.5%
Abnormality of the mouth -
Autosomal dominant inheritance -
Diffuse palmoplantar hyperkeratosis -
Esophageal carcinoma -
Parakeratosis -

Last updated: 12/1/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Sybert VP. Genetic Skin Disorders. New York: Oxford University Press; 1997;


Other Names for this Disease
  • Howel-Evans syndrome
  • Keratosis palmaris et plantaris with esophageal cancer
  • Keratosis palmoplantaris with esophageal cancer
  • TOC
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.