About oudtshoorn skin

What is oudtshoorn skin?

Erythrokeratolysis hiemalis is an extremely rare form of ichthyosis that was first described in South Africa but has subsequently been identified in other countries. In such cases, a link to South Africa has been determined. The disorder is characterized by periodic attacks of red (erythematous) plaques that are distributed equally on both sides of the body. A layer of skin can be peeled from these plaques. Symptoms usually improve with age. The disorder tends to become worse with the cold weather.



What are the symptoms for oudtshoorn skin?

KWE is characterized by the cyclical patchy Redness and thickening of the skin of the palms and soles, followed by the appearance dry Blisters which subsequently peel in an expanding pattern. The shedding skin has a thickish peel. The revealed surface skin appears glazed. These signs first appear during infancy or childhood and the disorder usually improves with age. The condition may be worsened by cold weather or episodes of fever. Secondary infection may complicate the condition. In some patients, slowly enlarging circular Red patches may develop, usually on the extremities. These slowly expand and have a trailing edge of peeling. Other frequently encountered associated symptoms include itching, excessive Sweating (hyperhidrosis or palmoplantar sweating) and a strong unpleasant odor.



What are the causes for oudtshoorn skin?

KWE is inherited and follows an autosomal mode of inheritance with males and females equally affected. It has been found to be associated with a duplication of an area of a chromosome that included an element known as an enhancer. This ‘switches on’ a nearby gene or genes. One of these appears to be the gene CTSB which is has been shown to be overexpressed. The protein produced by this gene is cathepsin B. This is an enzyme that plays an important role in proteolysis (breakdown of proteins) causing a major disruption to the epidermal cell’s normal growth and development. These damaged cells fail to mature properly and are pushed outwards, still retaining their nuclei and this forms the peel.

Furthermore, two different duplications have been discovered. The duplication found in the South African families is 7.67-kb in length while the duplication in Norwegian families is 15.93-kb. Both duplications overlap in the region of the enhancer. The genetic variation has not been determined for the families with KWE reported from Germany, Denmark or the USA.



What are the treatments for oudtshoorn skin?

There is currently no effective treatment for KWE. Topical steroids (anti-inflammatory preparation used to control many skin conditions) and retinoids (chemical compounds that are analogs of vitamin A) might help a little, but they can also aggravate it. Topical calciprotriol (a form of vitamin D) might also have minimal effect. Systemic steroids (anti-inflammatory derivatives of cortisol) have temporally resolved the circular lesions in a single patient. Measures to control sweating may be helpful. Photodynamic therapy has been used successfully in one patient.



What are the risk factors for oudtshoorn skin?

Keratolytic Winter erythema, also known as Oudtshoorn disease or Oudtshoorn skin is a rare autosomal dominant skin disease of unknown cause which causes redness and peeling of the skin on the palms and soles.

Risk factors

  • Onset, increased prominence and severity usually occur during winter.
  • It is a type of genodermatosis which is a condition caused by the person's genes not being suitable for a particular location.
  • This disease was first observed in Oudtshoorn in the western cape province of South Africa.
  • The disorder is characterized by periodic episodes of palmoplantar skin shedding preceded by redness and the appearance of dry superficial blisters. The peel is substantial and may be easily gripped.
  • The peeling is enhanced by exposure to water.
  • Symptoms may improve during pregnancy and with age. The disorder may worsen with cold weather and improve in the summer.
  • Affected Populations
  • In South Africa, families with KWE are scattered throughout the country and some families have emigrated to the UK and other countries. The condition is much more common in South Africa owing to the founder effect.
  • It affects families of both the white population and those of mixed racial descent.
  • KWE is inherited and follows an autosomal mode of inheritance with males and females equally affected.


Symptoms
Redness and peeling of the skin on the palms and soles
Conditions
Palmoplantar skin shedding preceded by redness,Appearance of dry superficial blisters
Drugs
Steroids and retinoid creams,Phototherapy in severe conditons



Is there a cure/medications for oudtshoorn skin?

Oudtshoorn skin, also known as Oudtshoorn disease, Keratolytic Winter erythema or is a rare autosomal dominant skin disease of unknown cause which causes redness and peeling of the skin on the palms and soles.

  • Onset, increased prominence and severity usually occur during winter.
  • It is a type of genodermatosis which is a condition caused by the person's genes not being suitable for a particular location.
  • This disease was first observed in Oudtshoorn in the western cape province of South Africa.
  • This disease affects people of white descent living in South Africa. Men are more affected by this condition. This condition worsens in the winter season compared to the summer
  • The disorder is characterized by periodic episodes of palmoplantar skin shedding preceded by redness and the appearance of dry superficial blisters. The peel is substantial and may be easily gripped.


Treatment

  • There is currently no effective treatment for Oudtshoorn skin
  • Topical steroids and other anti-inflammatory preparation used to control many skin conditions and retinoids might help a little, but they can also aggravate it.
  • Topical calcipotriol (a form of vitamin D) might also have minimal effect.
  • Systemic steroids like cortisol)have temporally resolved the circular lesions in many patients. However this has to be used only rarely
  • Measures to control sweating may be helpful.
  • Photodynamic therapy has been used successfully in the cure of this condition


Symptoms
Redness and peeling of the skin on the palms and soles
Conditions
Palmoplantar skin shedding preceded by redness,Appearance of dry superficial blisters
Drugs
Steroids and retinoid creams,Phototherapy in severe conditons



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