About familial continuous skin peeling

What is familial continuous skin peeling?

Peeling skin syndrome is a rare inherited skin disorder characterized by painless, continual, spontaneous skin peeling (exfoliation). Other findings may include blistering and/or reddening of the skin (erythema) and itching (pruritus). Peeling skin syndrome may clinically overlap with Netherton syndrome, which is also an autosomal recessive skin disorder belonging to the group of congenital ichthyoses, with onset in the neonatal period and infancy.



What are the symptoms for familial continuous skin peeling?

Ichythosis symptom was found in the familial continuous skin peeling condition

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. 



What are the causes for familial continuous skin peeling?

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. 



What are the treatments for familial continuous skin peeling?

Treatment is focused on managing the symptoms.



What are the risk factors for familial continuous skin peeling?



Is there a cure/medications for familial continuous skin peeling?

Skin peeling syndrome or familial continuous skin peeling happens on its own, is painless, and can last a lifetime while gradually getting better. Like skin peeling after a severe sunburn, affected people and/or their caretakers might frequently manually remove sheets of skin.

  • Applying skin-softening (emollient) ointments to treat peeling skin syndrome, particularly after a bath when the skin is moist, may provide some relief.
  • Vaseline or plain petroleum jelly are recommended.
  • None of the corticosteroids or systemic retinoids (derivatives of vitamin A) are recommended or effective, and all have the potential to have negative consequences.
  • Genetic counseling is advised for affected individuals and their families to explore the possibilities of genetic testing as well as the risk of having children with this condition.


Symptoms
Abnormal blistering of the skin,Abnormality of hair texture,Dry Skin,Aminoaciduria,Hyperhidrosis,Ichythosis
Conditions
Skin fragility,Lifelong peeling of the stratum corneum,Pruritus,Short stature,Easily removed anagen hair
Drugs
Ibuprofen,Naproxen



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