About erythrokeratolysis hiemalis
What is erythrokeratolysis hiemalis?
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 erythrokeratolysis hiemalis?
Shedding skin symptom was found in the erythrokeratolysis hiemalis condition
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 erythrokeratolysis hiemalis?
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 erythrokeratolysis hiemalis?
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 erythrokeratolysis hiemalis?
Erythrokeratolysis hiemalis is a rare disease associated with palms and soles. When you get affected by this disease, the skin of your palms and soles gets peeled. Itching and postulation happen if someone is affected by this disease. In most cases, the condition is present at the time of birth. But there is also a record of getting the disease during childhood or in adolescent period life.
Risk Factors of erythrokeratolysis hiemalis
- Risk factors of erythrokeratolysis hiemalis are epidermis with focal cellular edema and necrobiosis of the Malpighian layer.
- When clefting occurs in the stratum corneum, the necrobiotic Malpighian layer gets sandwiched in the hyperkeratotic stratum corneum.
- Erythrokeratolysis hiemalis is associated with chromosome 8p22-p23, which is found among the people of South Africa and Germany.
- Since it is an inherited skin disorder, people with the chromosome mentioned above easily get affected by the disease.
- The people residing in South Africa and Germany are at the highest risk of this disease.
- Aggravating factors may include: the winter season, excessive exposure to water, chemicals, or friction, illness, psychological stress, menstruation, secondary bacterial infection, certain medications including topical steroids and general anesthesia, and keratolytic winter erythema can improve in summer and during pregnancy.
Symptoms
Thickness and redness in the skin of palms and soles,Dry blister that cause the peeling of skin from the palms and soles,Shedding skin
Conditions
Epidermolysis bullosa simplex of hands and feet,keratolysis exfoliative
Drugs
Vaseline,Epaderm,Topical steroids and retinoids (to control many skin conditions),Topical calciprotriol,Systemic steroids (anti-inflammatory derivatives of cortisol),Photodynamic therapy
Is there a cure/medications for erythrokeratolysis hiemalis?
Currently, there is no effective treatment for Erythrokeratolysis hiemalis. Photodynamic therapy has shown disease-modifying results in one patient.
Treatment of the Disease
- No effective procedure of treatment is available. Although steroids and retinoids have been used, no fruitful result has been found.
- Topical steroids (anti-inflammatory preparation used to control many skin conditions) and retinoids 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.
Symptoms
Thickness and redness in the skin of palms and soles,Dry blister that cause the peeling of skin from the palms and soles,Shedding skin
Conditions
Epidermolysis bullosa simplex of hands and feet,keratolysis exfoliative
Drugs
Vaseline,Epaderm,Topical steroids and retinoids (to control many skin conditions),Topical calciprotriol,Systemic steroids (anti-inflammatory derivatives of cortisol),Photodynamic therapy
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