KRT1-related diffuse nonepidermolytic keratoderma

What is KRT1-related diffuse nonepidermolytic keratoderma?

A rare, genetic, isolated diffuse palmoplantar keratoderma characterized by diffuse, mild to thick, finely demarcated hyperkeratosis of palms and soles. Additional clinical findings include knuckle pad-like keratoses on fingers, hyperkeratosis of umbilicus and areolae, diffuse dry skin, hyperhidrosis, hangnails and frequent fungal infections. Histological examination of lesions reveals orthokeratotic hyperkeratosis, acanthosis, hypergranulosis, and mild lymphocyte infiltrations in the upper dermis with no evidence of epidermolysis.

Other condition names

  • KRT1-related diffuse NEPPK

Inheritance type

Autosomal dominant


  • Worldwide: Unknown

Age of Onset

  • Childhood
  • Infancy
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