Howel-Evans syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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.

Howel-Evans syndrome was described in 1996 as being identical with palmoplantar ectodermal dysplasia type III.

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.

References

• Howel-Evans W, McConnell RB, Clarke CA, Sheppard PM. Carcinoma of the oesophagus with keratosis palmaris et plantaris (tylosis): a study of two families Q J Med 1958;27:413-29. PMID 13579162.

External links

• Online Mendelian Inheritance in Man (OMIM) 148500
• Research into the genetic cause.
• Information about a number of keratodermas.