Acral peeling skin syndrome is a very rare genetic skin condition. It most commonly causes painless peeling of the top layers of skin on your hands and feet. Your arms and legs can also be affected.

As noted in a 2019 case letter, acral peeling skin syndrome was first reported in 1921 by Dr. Howard Fox. Since then, fewer than 100 cases have been reported in medical literature.

Although there’s no cure for acral peeling skin syndrome, symptoms are usually mild and don’t significantly alter your quality of life. The condition can usually be managed by applying ointments and creams to the affected areas.

Read on to learn everything you need to know about this rare genetic 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.

Here are some examples of how acral peeling skin syndrome affects the hands and feet:

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.

According to the Genetic and Rare Diseases Information Center, 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

Acral peeling skin syndrome isn’t contagious. It’s passed through families and is linked to gene mutations. Coming into contact with somebody with this condition doesn’t put you at risk of developing this condition.

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:

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.

A doctor or other healthcare professional may suspect acral peeling skin syndrome based on your symptoms, but diagnosis can be difficult because symptoms can resemble other conditions such as epidermolysis bullosa simplex, a condition that causes delicate skin.

To make a diagnosis, a doctor will consider your medical history and symptoms and will also perform a physical exam.

A confirmation of the diagnosis can be made by taking a biopsy of your skin for laboratory testing. A positive test for TGM5 or CSTA gene mutations suggests acral peeling skin syndrome.

You should see a doctor if you suspect that you or your child may have undiagnosed acral peeling skin syndrome.

Acral peeling skin syndrome is usually diagnosed in early childhood or at birth. It’s possible that it can remain undiagnosed in adulthood, such as in the case of a 44-year-old woman in a 2014 case study.

With a proper diagnosis, you’ll be best able to manage the condition.

Acral peeling skin syndrome is a very rare genetic condition that causes painless peeling of the skin on your hands and feet. It’s only possible to develop this condition if both your parents have a gene mutation associated with it.

Acral peeling skin syndrome doesn’t have a cure, but it rarely causes complications or affects your quality of life significantly. Symptoms can usually be managed by applying moisturizing ointments, lotions, or creams to affected areas.