Introduction

Peeling skin syndrome (PSS) is a rare autosomal recessive skin disorder where the outer layer of the skin (the stratum corneum) peels away continuously with or without trauma to the skin. It occurs when the usual gradual, invisible shedding of the stratum corneum is accelerated and causes the outermost layer and the underlying layers of the epidermis to separate.¹ This usually causes painless and spontaneous peeling of the superficial layers of the skin, but can also cause the formation of blisters, redness (erythema) and itching (pruritus). The symptoms are often exacerbated by external factors such as friction, heat, sweat, etc. The condition can present itself at birth or during early childhood and consists of two different subtypes, acral and generalised. While PSS is primarily a dermatological issue, its complications extend beyond the skin.

This article aims to explore PSS and the complications associated with it, including dermatological, systemic and emotional.

Subtypes of peeling skin syndrome

Generalised PSS

Generalised PSS tends to affect the skin over the entire body. It is further divided into two subtypes, inflammatory and non-inflammatory.

• Inflammatory: Skin peeling is associated with redness, itching and irritation and can have a similar appearance to eczema. This type of PSS carries a risk of infection and may cause discomfort due to the itching and irritation
• Non-inflammatory: Skin peeling isn’t associated with redness, itching or irritation and only carries a risk of infection if there is severe peeling. It is a milder form of the generalised PSS

Acral PSS

Acral PSS tends to be localised and only affects the extremities, such as hands and feet, but may also affect arms and legs. This can cause painful cracks and fissures on the palms and soles.

Causes

PSS is caused by genetic mutations which interfere with the proteins responsible for keeping the superficial layers of the skin intact, attached and functioning properly.

Different subtypes of PSS are linked to different gene mutations :

Generalised PSS

• CDSN gene : a nonsense mutation or a complete deletion of the CDSN gene, which encodes for corneodesmosin (CDSN) can cause generalised PSS.² Corneodesmosin is a protein found inthe stratum corneum and acts as an adhesion molecule to keep the skin cells (corneocytes) together and maintain skin integrity.³ In the absence of corneodesmosin, corneocytes begin to detach from each other, subsequently causing skin peeling/shedding

Acral PSS

• TGM5 gene: TGM5 gene encodes for the enzyme transglutaminase-5, which is responsible for maintaining epidermal integrity by forming cross-links between many different structural proteins within the cornified cell envelope (tough structure present beneath the cell membrane).⁴ Mutations in the TGM5 gene inhibit/reduce enzyme activity, which results in less cross-linking occurring between proteins, which subsequently reduces the integrity of the skin

PSS is most often inherited in an autosomal recessive pattern. That means a person needs two faulty copies of a relevant gene (one from each parent) to develop the condition. Parents who carry a single faulty copy (carriers) usually do not show symptoms but can pass the mutation to children.

Complications

Dermatological

Infections

PSS causes the superficial layers of skin to peel away and reduces the integrity of the skin, causing the skin barrier to weaken. Once the skin barrier has weakened, it fails to prevent pathogens such as bacteria, viruses and other antigens from entering the body, and the skin becomes more susceptible to infections. The infections can be bacterial (caused by bacteria such as Staphylococcus aureus), viral (such as cold sores), or they can be fungal (like athlete’s foot).

These infections can cause swelling, pain, discomfort and will often require the intervention of a medical professional and antibiotics/antivirals/anti-fungal treatments to help get rid of them. In some cases, infections can also spread across the body.

Inflammation

Some types of PSS, such as generalised inflammatory PSS, can cause the skin to become red and inflamed with chronic itching (puritus), mimicking the appearance of eczema. This may hinder the diagnosis of PSS. Inflammation can weaken the skin barrier, making the skin sensitive, dry and can cause discolouration.

Inflamed areas can often be tender to the touch, especially if the inflammation occurs on the skin in sensitive areas.

Inflammation may also cause excessive peeling and cracking, making the patient suffering with PSS more susceptible to infections.

Excessive transepidermal water loss

Excessive skin peeling and a weakened skin barrier will lead to an increased rate of transepidermal water loss (evaporation of water from within the skin into the environment), which can dry out the skin and increase people's chances of developing conditions such as psoriasis and atopic dermatitis.⁵ 

Systemic

Difficulties regulating temperature

People with PSS may struggle to regulate their body temperature and may become intolerant to harsher conditions, such as very hot weather or very cold weather, due to their weakened skin barriers.

Impact on hand and foot function

For people with certain forms of PSS, such as Acral PSS, hands and feet tend to be the most affected, which can cause difficulties in daily activities and practical life. Since the skin on the palms and soles is constantly under pressure, gripping and standing and walking, the peeling may get worse and form painful cracks and fissures. This can cause tasks involving grip (for example, opening a jar)to be harder to complete, and it can make walking and standing difficult, limiting mobility.

Hands and feet tend to be continuously exposed to moisture and friction, which, combined with the weakened skin barrier, can make these areas prone to infections.

Higher risk of allergies

People with PSS, especially those with the inflammatory type, tend to be at a higher risk of developing allergic conditions such as asthma, hayfever or atopic dermatitis. These allergies can further complicate the condition and may even exacerbate PSS symptoms further. It also adds more challenges to the daily lives of people suffering from PSS.⁶

Emotional

Visible skin peeling can cause a lot of emotional distress and may cause many people to become self-conscious and leading to low self-confidence. People can develop cosmetic concerns. This can sometimes lead to avoiding social activities or withdrawing from friends and classmates. Over time, the stress of managing a lifelong condition and worries about how others may react can contribute to anxiety or even depression. Emotional support from family, friends, and healthcare professionals is just as important as treating the skin itself.

When to see a doctor?

You should seek medical advice and get professional help if :

• Peeling is widespread or worsening
• There are signs of infection (fever, swelling, pus, spreading redness)
• The condition interferes with daily life, sleep, or emotional well-being

Key takeaways

Peeling Skin Syndrome (PSS) is a rare genetic condition that weakens the skin barrier, leading to continuous peeling, inflammation, and increased risk of infections. Depending on the subtype, it may also cause painful cracks on the hands and feet, difficulties with temperature regulation, and a higher likelihood of allergic conditions such as asthma or hay fever. Beyond the physical effects, PSS can also affect emotional well-being and self-confidence due to its visible symptoms.

FAQs

Q. Is peeling skin syndrome contagious?

A. No, peeling skin syndrome isn’t contagious. It is a genetic condition, which means that it can only occur as a result of spontaneous mutation or through inheritance.

Q. Is there a cure for peeling skin syndrome?

A. There is currently no cure for PSS, but supportive care and management can help improve the symptoms and improve daily life.

Q. Do the symptoms get worse with age?

A. Symptoms often begin in childhood. Some people find peeling improves as they grow, while others continue to have symptoms throughout life.