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PEELING SKIN SYNDROME (PSS)

Oheneba Kwakye Omane
Oheneba Kwakye Omane

How do you define 'peeling skin'?

Peeling skin is fortuitous damage to and loss of the upper layer of your skin (epidermis). Peeling skin can develop as a result of direct skin damage, such as sunburn or infection. It could also be an indication of an immune system problem or another illness. Peeling skin can cause rashes, irritation, dryness, and other irritating skin problems. Peeling skin can be caused by a variety of disorders, some of which are very serious. It's critical to receive a proper diagnosis as soon as possible.

What causes your skin to peel?

Your skin is constantly exposed to external factors that might irritate and harm it. Sun, wind, heat, dryness, and high humidity are all examples. Skin peeling can occur as a result of repeated irritation. It's very uncommon for newborns born over their due date to endure some painless skin peeling. Skin peeling can also be caused by an illness or condition that begins somewhere other than your skin.

Conditions that may cause general skin peeling include:

  • Allergic reactions
  • Infections, including some types of staph and fungal infections
  • Immune system disorders
  • Cancer and cancer treatment
  • Genetic disease, including a rare skin disorder, called acral peeling skin syndrome that causes painless peeling of the top layer of skin

Specific diseases and conditions that can cause peeling skin include:

  • Athlete's foot
  • Atopic dermatitis (eczema)
  • Contact dermatitis
  • Cutaneous T-cell lymphoma
  • Dry skin
  • Hyperhidrosis
  • Jock itch
  • Kawasaki disease
  • Medication side effects
  • Non-Hodgkin's lymphoma
  • Pemphigus
  • Psoriasis
  • Ringworm (body and scalp)
  • Scarlet fever
  • Seborrheic dermatitis
  • Staph infections
  • Stevens-Johnson syndrome
  • Sunburn
  • Toxic shock syndrome

What is then: Peeling Skin Syndrome (PSS)?

Peeling skin syndrome (PSS) is a group of rare inherited skin disorders in which the normal gradual process of invisible shedding of the outermost skin layers is hastened and/or aggravated. PSS is characterized by painless, continual, spontaneous skin peeling (exfoliation) due to a separation of the outermost layer of the epidermis from the underlying layers. Other findings may include blistering and/or reddening of the skin and itching. Symptoms may be present from birth or appear in early childhood and are often exacerbated by friction, heat or other external factors. Based on the extent of skin involvement, PSS may involve the skin of the entire body (generalized form) or is limited to the extremities, mostly hands and feet (localized form). Generalized PSS can be distinguished into an inflammatory type which is more severe, and a milder, non-inflammatory type. PSS may be caused by disease-causing variants in multiple genes encoding proteins with crucial functions for cell-cell adhesion.


Signs & Symptoms

Most forms of PSS manifest at birth or during infancy with shedding or peeling of the outermost layer of the skin. Skin peeling is spontaneous, painless, and may persist lifelong with gradual improvements. Often, affected individuals and their caregivers can remove sheets of skin manually, comparable to skin peeling after a severe sunburn. Other findings associated with this disorder may include blistering and skin fragility, itching, short stature, and/or newly formed hairs that can be plucked out more easily than normal. Skin peeling is often exacerbated by mechanical irritation of the skin, heat, sweat or water exposure or other external factors.

In the localized types, individuals develop blisters and erosions on hands and feet at birth or during infancy. The generalized inflammatory types may be associated with generalized inflammation of the skin (erythroderma) or localized thickened, red plaques (erythrokeratoderma), immuno-dysfunction, allergies, and susceptibility to infections, failure to thrive or metabolic wasting. In some patients, these disorders may be life-threatening, especially during the newborn period. Due to the variable clinical presentations of PSS, its often mild features and gradual improvement with age, PSS may be underdiagnosed and underreported.

Causes of Peeling Skin Syndrome

To date, genetic changes in several distinct genes have been reported to cause PSS. All known forms of peeling skin syndrome are inherited in an autosomal recessive pattern. Recessive genetic disorders occur when an individual inherits two abnormal copies of the disease gene, usually one from each parent. If an individual receives one normal gene copy and one abnormal gene copy for the disease, the person will be a carrier for the disease, but usually will not show symptoms. 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 for at least 4-5  abnormal recessive genes. Parents who are close relatives 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.

Diagnosis

A good history and physical exam are often sufficient to make the diagnosis, although specialized tests including surgical removal and microscopic evaluation (biopsy) of affected tissue may be necessary at times.

Treatment

Treating peeling skin syndrome by applying skin softening ointments, especially after a bath while the skin is moist, may offer some relief. Plain petroleum jelly or Vaseline is preferred.

To discuss the risk of having children with this disorder and the possibility of genetic testing, genetic counselling is recommended for affected individuals and their families.

References

. Alfares A, Al-Khenaizan S, Al Mutairi F. Peeling skin syndrome associated with novel variant in FLG2 gene. Am J Med Genet. Part A. 2017 Dec 173(12):3201-3204. PubMed PMID:28884927

. Alkhalifah A, Chiaverini C, Del Giudice P, Supsrisunjai C, Hsu CK, Liu L, Charlesworth A, McGrath JA, Lacour JP. PLACK syndrome resulting from a new homozygous insertion mutation in CAST. J Dermatol Sci. 2017 Nov 88(2):256-258. PubMed PMID: 28851602

. Bolling MC, Jan SZ, Pasmooij AMG, Lemmink HH, Franke LH, Yenamandra VK, Sinke RJ, van den Akker PC, Jonkman MF. Generalized Ichthyotic Peeling Skin Syndrome due to FLG2 Mutations. J Invest Dermat. 2018 Aug 138(8):1881-1884. PubMed PMID: 29505760

. Choe SJ, Kim BK, Lee S, Bak H, Lee JW, Ahn SK. A Case of Late-Onset Peeling Skin Syndrome Likely Triggered by Irritation. Ann Dermatol. 2017 Feb;29(1):119-120. DOI: 10.5021/ad.2017.29.1.119. Epub 2017 Feb 3. PubMed PMID: 28223764; PubMed Central PMCID: PMC5318512

. Rare Disease Database. (2021) Retrieved from  https://rarediseases.org/rare-diseases/peeling-skin-syndrome/

Oheneba Kwakye Omane

Health Enthusiast and Futurist