Keratose palmoplantaris diffusa circumscripta

Last Updated: 2019-08-26

Author(s): Anzengruber F., Navarini A.

ICD11: -

Trost 1880

Unna 1883

Körner 1901

  • Keratosis plamoplantaris diffusa 
  • Keratosis plamoplantaris diffusa Typ Vörner
  • Epidermolytische palmoplantare Hyperkeratose

Most common hereditary palmoplantar keratosis.


Autosomal dominant inheritance.

Mutation in the genes keratin 1 and 9.

Laterally blurred, erythematous, thick hyperkeratotic plaques covering the Palmae and Plantae.

There may also be isolated lateral blisters.

Nail changes are described.




Genetic analysis.

Hyperkeratosis, acanthosis.

  1. Das A, Kumar D, Das NK. KRT9 gene mutation as a reliable indicator in the prenatal molecular diagnosis of epidermolyticpalmoplantar keratoderma. Gene. 2014 Aug 1;546(1):124-8. 
  2. Das A, Kumar D, Das NK. Diffuse non-epidermolytic palmoplantar keratoderma.Indian Pediatr. 2013 Oct;50(10):979. 
  3. Hinterberger L, Pföhler C, Vogt T, Müller CS.Diffuse epidermolytic palmoplantar keratoderma (Unna-Thost-). BMJ Case Rep. 2012 Nov 9;2012.