Keratosis palmoplantaris diffusa circumscripta

Last Updated: 2023-07-07

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

ICD11: -

  • Palmoplantar keratoses

Trost 1880

Unna 1883

Körner 1901

  • Keratosis plamoplantaris diffusa 
  • Keratosis plamoplantaris diffusa type Vörner
  • Epidermolytic palmoplantar hyperkeratosis

Frequent hereditary palmoplantar keratosis.

1:100,000

Autosomal dominant inheritance.

Mutation in the keratin 1 and 9 genes.

Laterally indistinct, erythematous, thick hyperkeratotic plaques covering the palmae and plantae.

Sporadic blisters may also occur laterally.

Nail changes have been described.

Anamnesis.

Clinic.

Biopsy.

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.