- Howel-Evans syndrome
Name = PAGENAME
DiseasesDB = 33404
OMIM = 148500
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 predispositionto 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.
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.
* [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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См. также в других словарях:
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