Acral peeling skin syndrome (APSS) is an inheritable skin condition that causes painless peeling of the top layer of skin, typically on the hands or feet.
This condition is very rare, and a person’s biological parents must each carry a copy of the gene mutation for a person to show any symptoms.
This article covers what APSS is, how people inherit it, its symptoms, and how to treat it.
APSS is a skin condition.
According to the United States National Library of Medicine, people with this condition experience a painless peeling of the top layer of the skin.
The peeling typically occurs on the hands and feet. However, people may notice further peeling on the arms and legs.
Most people with the condition first start experiencing symptoms from birth. However, the condition can become evident during childhood or later in life.
This condition is very rare. However, because the symptoms of this condition are often very mild and may be similar to those of other skin conditions — such as epidermolysis bullosa simplex — doctors may be underdiagnosing it.
Epidermolysis bullosa simplex is a condition that causes the skin to become fragile and blister easily.
The U.S. National Library of Medicine state that APSS is a genetic condition that occurs due to a mutation in the TGM5 gene.
The TGM5 gene provides instructions for the body to make an enzyme called transglutaminase 5, which is a component of the epidermis, or the outer layer of the skin.
Transglutaminase 5 plays an important role in forming a structure called the cornified cell envelope, which envelopes the epidermis cells.
A TGM5 gene mutation reduces the production of transglutaminase 5. This makes the cornified cell envelope weaker and allows the top layer of the skin to separate very easily from the bottom layers.
People may notice that the peeling becomes worse when they are in a very moist environment or when they apply friction to their skin.
Inheritance pattern
A person can develop the condition if both biological parents carry a copy of the mutated gene. However, the biological parents will not typically show any symptoms of APSS themselves.
This inheritance pattern is an autosomal recessive pattern.
If a person carries one copy of the gene mutation, they are a carrier. Carriers do not tend to show any symptoms of this condition.
The main symptom of APSS is the painless peeling of the skin. Most people can remove this skin by hand, and it may come off in sheets, similar to peeling skin after a sunburn.
The National Organization for Rare Disorders (NORD) state that other symptoms may include:
- blistering
- itching
- easily removable hairs
If a person exposes their skin to heat, sweat, or water, they may notice that the peeling worsens.
According to the NORD, there is currently no cure for APSS.
However, some people may find that applying skin-softening ointments, such as petroleum jelly, may help them manage their symptoms.
This is most effective if a person applies the ointment after taking a bath, while the skin is still damp.
Doctors are unlikely to prescribe corticosteroids or retinoids for this condition. These are not effective for APSS and may cause adverse reactions.
Doctors will usually diagnose this condition after taking a person’s medical history and performing a physical exam.
According to one
A biopsy may involve surgically removing a part of the skin.
A person should see a doctor if they or their infant is showing any symptoms of APSS.
Although doctors cannot cure this condition, they can diagnose it and provide recommendations on how to treat it.
If a person is concerned about the risk of passing this condition to a future child, or if they would like to check to see if they are a carrier of the gene mutation, a doctor may be able to refer them for genetic testing.
Although this condition has no cure, people can manage the symptoms at home with ointments, such as petroleum jelly.
According to a 2016 case report, the condition has little or no “clinical repercussion.”
It is possible for a person with this condition to pass it on to their biological children if the other biological parent is a carrier or has APSS themselves.
APSS is a very rare genetic skin condition that causes painless shedding of the skin on the hands and feet.
People who have this condition often show symptoms from birth, but they can also appear later on.
People can only have this condition if both of their parents are carriers of the gene mutation. It is possible for a person with this condition to pass it on to their own biological children.
If a person believes that they may be a carrier for this condition, a doctor may be able to refer them for genetic testing.
Although there is currently no cure for APSS, people may find relief from the symptoms with the use of ointments, such as petroleum jelly. This is more effective if a person applies it after taking a bath, while the skin is still damp.