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.


Grease-replenishing care.

Acitretin 0.5 - 1 mg per kg bw daily. 


  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.