Peeling skin syndrome: Current status

Dermatol Online J. 2010 Mar 15;16(3):10.

Abstract

Peeling Skin Syndrome (PSS) is a rare genodermatoses characterized by asymptomatic, localized or generalized, continuous exfoliation of the stratum corneum; it may present at birth or in adulthood. We describe a patient having the type A non-inflammatory variant of PSS showing asymptomatic and continuous skin peeling from the neck, trunk, back, and extremities. Friction appeared to be an aggravating factor, but there was no seasonal variation. Histopathology in this condition reveals hyperkeratosis and splitting of the epidermis between the granular layer and the stratum corneum. No treatment for this disorder has been found to be effective so far.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Female
  • Homozygote
  • Humans
  • Hyperkeratosis, Epidermolytic / drug therapy
  • Hyperkeratosis, Epidermolytic / genetics
  • Hyperkeratosis, Epidermolytic / pathology*
  • Hypoproteinemia / pathology
  • Immunoglobulin E / blood
  • Mutation, Missense / genetics
  • Syndrome
  • Transglutaminases / genetics

Substances

  • Immunoglobulin E
  • transglutaminase 5
  • Transglutaminases