Orphanet: Non epidermolytic palmoplantar keratoderma

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

Non-epidermolytic palmoplantar keratoderma

Disease definition

A rare, isolated, diffuse palmoplantar keratoderma disorder characterized by diffuse, homogeneous, mild to thick, yellowish palmoplantar hyperkeratosis (sometimes spreading over the dorsal aspect of fingers), which presents a white spongy appearance following exposure to water, frequently associated with dermatophyte infections. Hyperhydrosis is usually present and skin biopsy shows non-epidermolytic changes.


Classification level: Disorder
  • Synonym(s):
    • Autosomal dominant diffuse palmoplantar keratoderma, Norrbotten type
    • Diffuse palmoplantar keratoderma, Bothnian type
    • NEPPK
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Infancy, Childhood
  • ICD-10: Q82.8
  • OMIM: 600231
  • UMLS: C1833030  C1838359
  • MeSH: -
  • GARD: 1862
  • MedDRA: -
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.