About keratolytic winter erythema

What is keratolytic winter erythema?

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 keratolytic winter erythema?

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 keratolytic winter erythema?

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 keratolytic winter erythema?

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 keratolytic winter erythema?

Keratolytic winter erythema is distinguished by cyclical patchy redness and skin thickening of the palms and soles, which is followed by the formation of dry blisters that peel in an extending pattern. A thickish peel covers the losing skin. The exposed surface skin looks to be glazed. These symptoms normally occur during infancy or youth, and the disease improves with maturity.

Risk Factors

  • KWE is inherited in an autosomal dominant manner, with males and females equally afflicted. It was discovered to be linked to the duplication of a chromosomal region containing an enhancer element.
  • One in every 7,200 white Afrikaans speakers is thought to have keratolytic winter erythema.
  • It is passed down in a monogenic autosomal dominant manner with strong penetrance but variable expressivity.
  • It is most commonly found in childhood or early adulthood.


Among the aggravating factors are:

  • The winter months
  • Excessive water, chemical, or friction exposure
  • Illness
  • Stress on the mind
  • Menstruation
  • Bacterial secondary infection
  • Topical steroids and general anaesthesia are examples of drugs.


Symptoms
Redness of skin or mucous membrane,Abnormal excessive perspiration,Skin pustules
Conditions
Erythema,Hyperhidrosis,Pustule
Drugs
NA



Is there a cure/medications for keratolytic winter erythema?

Keratolytic winter erythema is distinguished by cyclical patchy redness and skin thickening of the palms and soles, which is followed by the formation of dry blisters that peel in an extending pattern.

  • A thickish peel covers the losing skin.
  • The exposed surface skin looks to be glazed.
  • These symptoms normally occur during infancy or youth, and the disease improves with maturity.
  • KWE is inherited in an autosomal dominant manner, with males and females equally afflicted.
  • It was discovered to be linked to the duplication of a chromosomal region containing an enhancer element.


Treatment

  • KWE presently has no viable treatment.
  • Topical steroids (anti-inflammatory medications used to treat a variety of skin disorders) and retinoids (chemical compounds that are vitamin A analogues) may assist temporarily, but they can also aggravate the condition.
  • Calciprotriol (a kind of vitamin D) used topically may also have no effect.
  • Systemic steroids (cortisol anti-inflammatory compounds) temporarily cleared the circular lesions in a single case.
  • Sweating management measures may be beneficial.
  • In one case, photodynamic treatment was employed successfully.


Symptoms
Redness of skin or mucous membrane,Abnormal excessive perspiration,Skin pustules
Conditions
Erythema,Hyperhidrosis,Pustule
Drugs
NA



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