Howel-Evans syndrome

Howel-Evans syndrome

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DiseasesDB = 33404
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OMIM = 148500
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Howel-Evans syndrome is an extremely rare condition in which the skin of the palms of the hands, and soles of the feet, are affected (hyperkeratosis). The effects on the palms and soles is called tylosis, and in Howel-Evans syndrome, there is a predisposition to oesophageal cancer, particularly squamous cell carcinoma.cite journal |author=HOWEL-EVANS W, McCONNELL RB, CLARKE CA, SHEPPARD PM |title=Carcinoma of the oesophagus with keratosis palmaris et plantaris (tylosis): a study of two families |journal=Q. J. Med. |volume=27 |issue=107 |pages=413–29 |year=1958 |pmid=13579162 |doi= |url=http://qjmed.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=13579162]

Howel-Evans syndrome was described in 1996 as being identical with palmoplantar ectodermal dysplasia type III.It is a rare autosomal dominant disorder with genetic abnormality at chromosome 17q25.

Treatment

The skin is treated with a keratolytic (such as salicylic acid) to dissolve the excess skin, and systemic treatment with retinoids (derivatives of Vitamin A) may be given.

ee also

* Palmoplantar keratoderma

References

External links

* [http://www.liv.ac.uk/luds/resrch/mgo/tylosis.htm?..&..&people&cds&od&jkf&home.htm Research into the genetic cause.]
* [http://www.thedoctorsdoctor.com/diseases/keratodermas.htm Information about a number of keratodermas.]


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