Peeling skin syndrome

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Disease Overview

Peeling skin syndrome (PSS) refers to a group of conditions that causes skin to peel and tear easily. The main symptom is continual peeling of the skin. The peeling skin is usually painless. Additional symptoms may depend on the form of PSS. Some people with PSS have itching, redness, and scarring. The symptoms of PSS can appear anytime from birth to adulthood, but usually occur by childhood. There are two forms of PSS. In the generalized form, skin peeling involves most of the body. The generalized form has two subtypes, noninflammatory and inflammatory. There is an acral form in which skin peeling is limited to the hands and feet. PSS is caused by genetic variants in several different genes and is inherited in an autosomal recessive pattern of inheritance. PSS is diagnosed based on the symptoms. Other more common conditions may need to be excluded. Treatment is focused on managing the symptoms.[10180][15811][15814] 


Synonyms

  • Keratolysis exfoliativa congenita
  • Familial continuous skin peeling
  • Skin peeling syndrome
  • Deciduous skin
  • Familial continuous skin peeling syndrome
  • Idiopathic deciduous skin
  • Keratosis exfoliativa congenita
  • Peeling skin disease
  • PSS

For more information, visit GARD.

National Organization for Rare Disorders