Ichthyosis

• Hyperkeratosis

introduction

Ichthyosis is what is known as fish scale disease.

This disease is based on a genetic defect which can be passed on, but can occasionally also occur in people without a genetic defect.

About every 300th person is affected by ichthyosis, some less severely, others, however, very pronounced. Ichthyosis is an incurable skin disease. Nevertheless, there is a lot that can be done about cornification and drying out of the skin, both symptoms of ichthyosis.

It is also important that ichthyosis is not a contagious disease and that no special precautionary measures need to be taken in physical contact with other people.

causes

In order to better understand the cause of ichthyosis, one should first consider the structure and the regeneration mechanism of the skin understand in its main features:

In the skin is it about that largest organ of the human body, which is constantly renewed and is therefore able to take away from the outside Wounds like cuts or other injuries within a few days to weeks to heal.
In addition, the skin consists of several layers:
The lowest layer of skin is the so-called Basal layer (stratum basale). Here new skin cells are constantly being formed, which then migrate up through different layers of skin and finally through different ones Deposits (including keratin) keratinize. The keratinized cells are the cells furthest up in our skin and have already died. Through this Horny layer it ensures that no water penetrates the skin, even when we go swimming and shower, and the horny layer takes care of the Firmness of the skin.
Within 4 weeks, a skin cell migrates from the basal layer through the entire skin layer and then ends in the horny layer and is repelled from here and falls off unnoticed, for example when showering.

In ichthyosis, the balance of the skin, which normally ensures that roughly the same number of cells are formed as are rejected, is disturbed. The cause of ichthyosis is thus one Disruption of new skin formation.

It will so many new skin cells that produces it constantly to one Excess of keratinized cells comes on, which causes it to settle on the skin Dander form what ichthyosis ultimately gets its name as Fish scale disease owes.
Depending on which form of ichthyosis it is, the Inheritance should be considered as the cause of the development of ichthyosis. For example, the vulgar ichthyosis autosomal dominant inherited. This means that one sick parent is enough to pass the disease on. Other forms, however, will be recessive inherited, which means that a sick parent does not have to cause the child to suffer from the condition too.

The cause of ichthyosis is generally always one “Too much” of keratinized, dead skin cells on the skin surface. On the one hand, this can be due to the fact that too many cells are being produced and, on the other hand, it can be due to the fact that too few cells are rejected on the surface and too many keratinized cells accumulate on the surface of the skin despite normal production.

diagnosis

In order to be able to diagnose ichthyosis, it is usually best to have one Dermatologist (dermatologist) to seek out because this is on Skin disorders specialized and therefore has the most experience in this area.

The diagnosis of ichthyosis is often one for an experienced dermatologist Eye diagnosis, On the other hand, it can sometimes be difficult for others to choose between an ichthyosis and an Neurodermatitis to distinguish, especially when it comes to one mild form of ichthyosis acts.

As with the various forms of ichthyosis, however, usually one specific infestation pattern of the body is present, a visual diagnosis is possible.
In addition, the patient can have a small Tissue sample can be removed. These tissue samples are then collected using a Microscope analyzed, one calls this histological examination. Here it can be determined whether the Keratinization process of the skin is disturbed, which is the case with ichthyosis.
Furthermore, the patient can blood be removed, which then the DNA analysis, so the Evaluation of the genetic material serves. This can be used to determine whether it is actually a Genetic defect acts, which then leads to the disease. Since ichthyosis often occurs in the first few months of life, the Pediatrician be consulted.

Frequency distribution

The ichthyosis is not a rare disease.
The mild form of ichthyosis occurs in around one in 300 people. The more severe forms, on the other hand, are rarer.
However, patients should not be ashamed of the disease, but learn to live with it and accept it as part of themselves.

Symptoms

The symptoms of ichthyosis are very different depending on the severity and depending on the form of ichthyosis.

However, it is typical for all forms that there is one in ichthyosis very thickened top layer of skin comes. In addition, the skin flakes itself off and more dandruff than in patients without ichthyosis.
Often comes a itching and the skin may also be reddened. Especially in the cold Winter months it comes to one Reinforcement the symptoms, since the skin loses moisture in healthy people during the cold season, which is particularly noticeable in patients with ichthyosis. In rare cases it occurs Blistering on the skin.

The more severe forms of ichthyosis are shortly after the birth recognizable, while on the other hand mild forms especially in the winter months noticeable and are sometimes almost completely asymptomatic in summer.

It is important to know that through the In the corneal layer of the skin, hardly any heat can escape can, which leads to patients with ichthyosis sweat less as healthy patients. However, this has the disadvantage that the Thermoregulation of the body is disturbed by it and when Sports the body more water added otherwise the body will overheat and dry out.

therapy

Ichthyosis is a disease in which no therapy for a complete cure leads.

Still, there are several ways to relieve symptoms of ichthyosis:
It is important that skin to keep supple and the horny layer of the skin by using Keratolytics to solve.
At Keratolytics are ingredients that are often found in special creams are included, these ensure that the topmost horny layer, which too a lot of keratin contains, is replaced.
Furthermore, the patient must at least bathe once a day and then the skin with a Moisturizer, Which Ureas should contain lotion.

Especially that salty water the dead sea seems to have a particularly good effect on that skin of patients, why should they? Baths with dead sea salt recommended.

All of these measures alleviate the symptoms, however, cannot remedy the actual cause, the genetic defect.
In addition, the cornea can be treated every day with baths and ointments, but it is also continually reproduced. Ichthyosis is thus a disease that affects the patient a life long will accompany. Therefore also can Support groups as an important part of therapy, help any that may arise Worries and fears to cope with the patient and to show that he is not alone, but that there are many people with such a disease.

prophylaxis

Since ichthyosis is based on a genetic defect, there is no possibilities of prophylaxis.

Still, it is important, especially in patients who only have a mild form of ichthyosis, to keep the skin clean to take care of sufficientlywhen the ichthyosis does not break out as badly as it does in winter.

forecast

Ichthyosis is still one incurable Disease, the symptoms of which can only be alleviated.

However, since quite a large number of patients are affected by the disease, there are different ones Research approacheshow to use gene therapy measures may continue to have a positive effect on the symptoms of ichthyosis in the future.

Furthermore, there is the possibility that patients with a severe form of ichthyosis develop a milder form during puberty, although it must be said at this point that the opposite scenario is also possible.

In general, ichthyosis limits the Life expectancy of a patient, however, by no means a symptom-relieving therapy is an almost complete normal life possible.

Further topics from this area

Hyperkeratosis

Hyperkeratosis leads to increased cornification of the skin. Various causes can be responsible for the increased corneal formation.
Here you get to the topic: Hyperkeratosis