Acral peeling skin syndrome is a genetic skin disorder characterized by painless peeling of the top layer of skin. "Acral" refers to the fact that the peeling is most apparent on the hands and feet, although peeling may also occur on the arms and legs. The peeling is usually present from birth, but can appear later in childhood or early adulthood. Acral peeling skin syndrome can be caused by mutations in the TGM5 gene. Mutations in the CSTA gene have also been linked to this condition. It is inherited in an autosomal recessive pattern. There is no cure for acral peeling skin syndrome. Treatment is aimed at treating the symptoms present in each individual.

TGM5 gene. This gene provides instructions for making an enzyme called transglutaminase 5, which is a component of the epidermis (the outer layer of skin). Transglutaminase 5 plays a critical role in the formation of a structure called the cornified cell envelope, which surrounds epidermal cells and helps the skin form a protective barrier between the body and the environment.

TGM5 gene mutations reduce the production of transglutaminase 5 or prevent cells from making any of the protein. A shortage or absence of this protein weakens the cornified cell envelope, allowing the outermost cells of the epidermis to separate easily from the underlying skin and peel off. This peeling is most noticeable on the hands and feet because these areas are more frequently exposed to moisture and friction.

More recently, recessive loss of function mutations in the CSTA gene have been linked to this condition.

Acral peeling skin syndrome is characterized by painless peeling of the top layer of skin (the epidermis). The term "acral" refers to the fact that the peeling is most apparent on the hands and feet. Occasionally, peeling may also occur on the arms and legs. Symptoms typically develop soon after birth, but may also begin in childhood or early adulthood. Skin peeling is made worse by exposure to heat, humidity and other forms of moisture, and friction. The underlying skin may be itchy and red, and in rare cases, blisters may form. The affected areas typically heal without scarring. Acral peeling skin syndrome is not associated with any other health problems and generally doesn't significantly impair quality of life.

Additional treatment information and tips for daily living are available through the Information Leaflet for Parents/Carers which was developed by the Birmingham Children's Hospital NHS Foundation Trust.