Erythrokeratoderma is a disease of the skin, which belongs to the keratoderma group. It is a disease in which there is a thickening of the outermost layer of the skin, as well as skin redness. This thickening of the skin is also called keratinization or hyperkeratosis and the redness of the skin is erythroderma.
What is erythrokeratoderma?
Erythrokeratoderma belongs to the subgroup of hereditary palmoplantar keratosis. This skin disease, in turn, belongs to the keratoderma family. Erythrokeratoderma is divided into four other diseases:
These are erythrokeratodermia progressivia symmetrica, erythrokeratodermia variabilis, erythrokeratodermia papillaris et reticularis and erythrokeratodermia en cocardes Degos. In erythrokeratoderma, there is keratinization of the skin on the hands as well as the feet along with skin redness due to inflammation. These skin rednesses are also called erythroderma. Erythroderma can also occur independently of erythrokeratoderma or as a subsequent condition in other dermatological diseases. The skin is the protective barrier of humans against external influences from the environment such as UV rays. The skin consists of several layers, the outer layer being formed by the epidermis. This in turn is formed by other layers. The innermost layer is the basal layer, also called stratum basale. Further out follow the prickle layer or stratum spinosum, the granular layer or stratum granulosum, the shiny layer or stratum lucidum and finally the outermost layer, which is the horny layer or stratum corneum. The epidermis consists of a specific type of cell, the keratinocytes. They produce keratin and keratinize as they migrate from the basal layer to the stratum corneum. In the process, these cells die. The stratum corneum consists of dead and keratinized keratinocytes, which are then called horny cells or corneocytes. A disturbance in this process can lead to increased keratinization of the skin. There is an increased production of corneocytes, which accumulate in the cornea and lead to thickening of this skin layer. This process is called hyperkeratosis. In erythrokeratoderma, there is also erythroderma, the redness of the skin due to inflammation.
Causes
Erythrokeratoderma, like most keratoderma diseases, is hereditary. However, it can also occur as a concomitant of other diseases. Erythrokeratoderma may develop if the affected individual suffers from other conditions such as ataxia, congenital nonbullous ichthyosiform erythroderma, hypotrichosis-deafness syndrome, or bullous congenital ichthyosiform erythroderma. There is an increased formation of corneocytes, which are deposited in the cornea, or the body is no longer able to renew the cornea naturally. Erythroderma is divided into primary and secondary erythroderma. Primary erythroderma occurs when the affected person previously suffered from another disease. Then it is divided into acute or chronic erythroderma. Acute erythroderma needs quick treatment, otherwise it can lead to a life-threatening situation. Chronic erythroderma is less aggressive than the acute form and is inherited as in the case of erythrokeratoderma. Secondary erythroderma is the form which is formed due to previous dermatological diseases. This is the most common form of erythroderma.
Symptoms, complaints, and signs
Erythrokeratoderma is characterized by two main underlying diseases. Hyperkeratosis, the keratinization of the skin on the hands and feet, occurs. This occurs due to the formation of scales. In addition, erythroderma occurs. Erythroderma refers to an inflammation of the skin together with a dilation of the vessels. This causes the visible reddening of the skin in erythrokeratoderma. Depending on the severity of the erythroderma, there may be severe loss of fluids as well as nutrients such as salts. Due to the dilated vessels, there is also an increased loss of body heat to the environment. The result is increased sensitivity to cold temperatures.
Diagnosis and course
The diagnosis of erythrokeratoderma is made by a dermatology specialist.The cornifications of the hands and feet as well as the reddening of the skin due to inflammation and vasodilatation are clearly visible. If erythrokeratoderma is not treated, the cornifications may spread. A continuous increase of the cornification can also occur, so that the mobility of the hands and feet can be impaired. If erythroderma is also not treated, there can be a severe loss of fluid as well as trace elements, which can cause extensive damage to the body. It can also lead to a life-threatening situation for the affected person.
Complications
In erythrokeratoderma, the patient primarily suffers from cosmetic complications due to severe thickening of the skin with redness. Due to these symptoms, there may be decreased self-esteem and also inferiority complexes. Likewise, when erythrokeratoderma is pronounced, there is loss of nutrients and fluids. In the process, the affected person may also lose body heat and thus suffer from cold extremities. Similarly, temperature sensitivity increases, which can lead to uncomfortable feelings and pain on the skin, especially in cold temperatures. Without treatment, very high losses of important nutrients and trace elements occur. These losses can damage the body in the long run and have a negative impact on health. However, the exact complications depend on the amount of loss. As a rule, erythrokeratoderma can be treated relatively well with the help of medication to limit the symptoms. There are no further complications. If early treatment is given, life expectancy is not reduced. However, if the body continues to lose fluids and nutrients, the cardiovascular system may be damaged.
When should you see a doctor?
A visit to the doctor is necessary when there are unusual changes in the appearance of the skin. Thickening of the upper layer of the skin should be medically clarified to determine the cause. If sensory disturbances occur or there is numbness, a doctor should be consulted. The formation of scales on the skin indicates dehydration. This should be treated medically, as it can lead to feelings of tension and cracking. In case of open wounds, germs can enter the organism and cause further diseases. In severe cases, there is a risk of blood poisoning. A doctor should be consulted as soon as further complaints develop or pain occurs in the affected areas. Reddening of the skin or the loss of body fluids through the skin should be clarified by a doctor. If the temperature sensitivity increases or the hands and feet cool down unusually quickly, these symptoms should be presented to a doctor. If there are changes in the gait due to the complaints or if there is a loss of strength, a visit to the doctor is necessary. In case of a general feeling of indisposition, a feeling of illness as well as a spreading of the affected region, a doctor should also be consulted. If the affected person suffers emotionally or mentally from the visual blemish, it is advisable to talk to a medical professional about various options for relief.
Treatment and therapy
Hyperkeratosis can be treated with the help of keratolytics by a dermatologist. Keratolytics include benzoyl peroxide, azelaic acid, alpha-hydroxy acids, urea, and retinoids. They soften the cornea. After that, it can be removed with various tools such as pumice stone or callus rasp. Erythroderma is treated with glucocorticoids. This treatment is essential, otherwise dehydration and excessive stress on the cardiovascular system may occur. In addition, the patient is given sufficient fluids to counteract the erythroderma.
Outlook and prognosis
The hereditary disease can only be treated symptomatically by medical professionals. Because physicians are not allowed to alter a person’s genetics for legal reasons, no complete cure is possible at the present time. However, the symptoms can be significantly alleviated when medical care is sought. Therapy takes place through the administration of medications. The agents soften the cornea so that manual and independent removal of the unwanted tissue can take place. Without treatment, there is a long-term loss of nutrients and trace elements through the skin.This deprives the organism of vital elements and a deficiency syndrome occurs. The prognosis deteriorates considerably under these conditions. There is a risk of cardiovascular stress and dehydration may occur. Thus, a potentially life-threatening condition exists for the affected person. With medical care, this development can be virtually eliminated. Due to the removal of the cornea as well as an adequate supply of nutrients to the skin, the loss of nutrients is contained. Long-term consequences are not expected under these conditions. The care of the skin is carried out in a lifelong therapy. As soon as this is under- or interrupted, the callus develops within a short time. Thus, there is a relapse of symptoms and a worsening of the prognosis.
Prevention
Erythrokeratoderma is a hereditary disease. Therefore, there are no preventive measures. Once the disease appears, it must be treated to prevent secondary damage.
Aftercare
In most cases of erythrokeratoderma, the patient has very limited options for aftercare. The affected person is therefore dependent on treatment by a medical professional, whereby first and foremost a rapid and above all early diagnosis of the disease is important in order to prevent further complications. The earlier the erythrokeratoderma is detected, the better the further course of the disease usually is. Patients with erythrokeratoderma are dependent on taking medications that can permanently alleviate the symptoms. Care must be taken to ensure that the medication is taken correctly, and the patient should always discuss interactions and side effects with a doctor. In cases of doubt, a doctor should always be consulted. Furthermore, the patient should always take care of his body and not put it under unnecessary strain. Taking plenty of fluids can also have a positive effect on the course of erythrokeratoderma and prevent further complaints. In most cases, the life expectancy of the patient is not reduced by this disease. Contact with others affected by the disease may also prove useful, as this often results in an exchange of information.
Here’s what you can do yourself
The hereditary disease offers patients little scope for self-help. On a day-to-day basis, self-help measures should be understood as support to the conventional medical guidelines, which are intended to improve well-being. Patients suffering from erythrokeratoderma can improve their skin quality with a coordinated skin care regimen and thus alleviate some of their complaints. Creams and ointments that have a high fluid content are particularly recommended. In addition, there are special fluids that can be used in case of rapid callus formation. If possible, the care products should be applied several times a day to the desired areas of the skin. At the same time, avoid wearing synthetic garments. These dry out the skin and lead to an increase in the symptoms. During stays in swimming pools or public baths, the feet should be cared for especially intensively, as the chlorine contained in the water also dries out the skin. Using various rasps or stones to remove calluses, the patient can remove the dead skin layers independently. Medicinal foot baths also support the skin and prevent the formation of cracks. These can be carried out independently as required. The entire body should be protected from dehydration. For this purpose, the recommended amount of fluid should be taken daily.
