What is the definition of Peeling Skin Syndrome?

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. 

What are the alternative names for Peeling Skin Syndrome?

  • 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

What are the causes for Peeling Skin Syndrome?

Peeling skin syndrome is caused by the TGM5, CSTA, CHST8, SERPINB8, FLG2, or CDSN gene not working correctly. There may be other unknown genes involved as well. DNA changes known as pathogenic variants are responsible for making genes work incorrectly or sometimes, not at all.

What are the symptoms for Peeling Skin Syndrome?

The following list includes the most common signs and symptoms in people with peeling skin syndrome (PSS). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition. Symptoms may include:
  • Continuous peeling of the outer layer of the skin
  • Itching
  • Darkened skin patches (hyperpigmentation)
  • Redness
Symptoms can begin at any age, but usually begin in childhood. In some types of peeling skin syndrome, the skin peeling is painless and there are few, if any, additional symptoms. Some people develop scarring around the areas of skin peeling. The area of the body involved depends on the type of PSS.

What are the current treatments for Peeling Skin Syndrome?

Treatment for peeling skin syndrome is focused on managing the symptoms. Skin softening ointments and plain petroleum jelly have been used by some individuals. Keratolytic agents which remove excess skin can help speed up shedding and improve appearance. People with peeling skin syndrome may be treated by a dermatologist.

Is Peeling Skin Syndrome an inherited disorder?

Peeling skin syndrome is inherited in an autosomal recessive pattern. All individuals inherit two copies of each gene. Autosomal means the gene is found on one of the numbered chromosomes found in both sexes. Recessive means that both copies of the responsible gene must be altered to have the condition.   People with autosomal recessive conditions inherit one alteration from each of their parents. The parents, who each have one gene alteration, are known as carriers.  Carriers of an autosomal recessive condition typically do not have any signs or symptoms (they are unaffected). When two carriers of an autosomal recessive condition have children, there is a 25% (1 in 4) chance to have a child with the condition.

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