About skin peeling syndrome

What is skin peeling syndrome?

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 skin peeling syndrome?

Painless peeling symptom was found in the skin peeling syndrome condition

Acral Peeling skin syndrome causes painless peeling of the top layer of your skin called the epidermis. Peeling usually affects your hands and feet but may also affect your arms or legs. The word “acral” refers to a condition that affects your peripheral body parts.

Other symptoms can include easily plucked hairs and fragile skin. The skin under the Peeling skin may be Itchy and red, but the condition rarely causes Blisters or scarring.

Peeling generally starts from birth but can also appear in late childhood or early adulthood. In a 2016 case study, a child with acral Peeling skin syndrome developed blisters and peeling on his palms and soles at the age of 6 months.

Peeling tends to get worse after exposure to:

  • heat
  • humidity
  • moisture

Acral Peeling skin syndrome isn’t associated with any other health conditions and usually doesn’t significantly alter quality of life.

What are the causes for skin peeling syndrome?

Acral peeling skin syndrome has been linked to mutations in the gene transglutaminase 5 (TGM5) and has less commonly been linked to mutations in the gene cystatin A (CSTA).

TGM5 is responsible for telling your body to create the TGM5 enzyme, which is found in many tissues, including your epidermis. The TGM5 enzyme helps create the cornified cell that acts as a protective barrier between your skin and the outside world.

If you don’t produce the TGM5 enzyme, the outermost layer of your skin can easily separate and peel off. Your hands and feet tend to be most affected because they’re the parts of your body most exposed to moisture and friction.

The gene CSTA codes for the CSTA enzyme, which plays a role in cell to cell adhesion.

Inheritance pattern

Gene mutations linked to the development of acral peeling skin syndrome are inherited in an autosomal recessive pattern. To develop symptoms, you need to receive one copy of the mutated gene from both of your parents.

If you only receive a gene mutation from one parent, you’ll be a carrier of the gene mutation but won’t develop symptoms. Your parents don’t need to have symptoms to be carriers.

If both parents have the gene mutation:

  • a child has a 25 percent chance of developing symptoms
  • a child has a 50 percent chance of carrying the gene mutation but not developing symptoms
  • a child has a 25 percent chance of neither carrying the gene mutation nor developing symptoms

What are the treatments for skin peeling syndrome?

Treatment for acral peeling skin syndrome revolves around managing your symptoms.

Is there an acral peeling skin syndrome cure?

There’s no cure for this condition, although it rarely affects quality of life significantly or leads to complications.

Acral peeling skin syndrome home remedies

Symptoms are often manageable at home with a type of moisturizer called an emollient. Emollients soothe and soften dry or flaking skin by covering your skin with a thin, oily layer that helps seal in moisture. They come in a variety of forms, such as:

  • ointments
  • sprays
  • creams
  • lotions

Some of the most popular emollients include:

  • shea butter
  • cocoa butter
  • petroleum jelly
  • beeswax
  • mineral oil
  • coconut oil
  • jojoba oil
  • olive oil

You may also be able to minimize your symptoms by avoiding:

  • immersion in water
  • hot temperature
  • high humidity
  • friction on your hands or feet

Medical treatment

Medical treatments usually aren’t necessary. If you develop blisters, a doctor or other healthcare professional can lance them for you with a sterile needle to drain the fluid. They can also apply a light dressing to help you avoid an infection.

What are the risk factors for skin peeling syndrome?

Peeling skin syndrome due to variants in the CHST8 and CSTA genes were reported in consanguineous Pakistani and Bedouin families, respectively. In Japanese individuals, a particularly common deletion involving CDSN has been reported. In Europeans, acral PSS due to TMG5 variants is more common.

The risk for two carrier parents to both pass the altered gene copy and, therefore, have an affected child is 25% with each pregnancy. The risk to have a child who is a carrier like the parents is 50% with each pregnancy. The chance for a child to receive normal genes from both parents is 25%. The risk is the same for males and females.

All individuals are thought to be carriers of at least 4 to 5 abnormal recessive genes. Parents who are close relatives (consanguineous) have a higher chance than unrelated parents to both carry the same abnormal gene, which increases the risk to have children with a recessive genetic disorder.

Is there a cure/medications for skin peeling syndrome?

A genetic disease caused due to mutations mainly caused due to mutation in the TGM5 gene is called Skin Peeling Syndrome.

  • TGM5 enzymes act as a protective barrier for the outermost layer of the skin
  • Scarcity in the production of TGM5 enzyme give rise to the separate layer and peeling off.
  • Light peeling or dryness is common in people
  • If it is happening continuously, it is a syndrome and needs attention.

Treatment for severe development of infection or blisters (boils with fluids) is required as follows:

  • This drains the fluid
  • Application of light dressing
  • To avoid infection if it is left uncovered
  • Doctor lance them with a sterile needle

Post the confirmation of skin peeling through a skin biopsy, physical examination, and medical history of a patient; home remedies are required to start. As there is no specific medical cure for the disease:

  • Ointments
  • Sprays
  • Lotions

A few of the great examples of emollients are:

  • Beeswax
  • Olive oil
  • Coconut oil
  • Shea butter

In addition to that, some preventions can help taking care in a better way by minimizing:

  • Exposure in hot temperatures
  • Immersion in water
  • Friction on hands and feet
  • High humidity environments

Peeling Skin,Sunburned Skin like appearance,Fragile Skin,Painless Peeling,Skin affected on Peripheral body parts
Plucked Hairs,Itchy Skin,Red Skin,Blisters,Scarring
Petroleum Jelly,Skin-Softening Ointments,Corticosteroids,Retinoids

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