Ichthyosis (ichthyosis) belongs to the skin diseases, which is noticeable by increased scaling. Different forms of this disease exist, which differ from each other.
What is ichthyosis?
Behind the name ichthyosis (ichthyosis) is a hereditary skin disease, which is associated with a strong, diffuse cornification disorder. This often affects the entire skin. Occasionally, the disease is also called fish scale disease. It exists in different forms. In exceptional cases, ichthyosis is part of a syndrome. In independence of the form of the disease, this disease is not contagious. Which form of ichthyosis the person suffers from is defined according to the type of inheritance. Apart from that, the different forms show different signs as well as different fine tissue appearances on the skin. Furthermore, transitional forms occasionally occur. The symptoms of the disease usually exist since birth. Sometimes, however, they develop only in the first years of life. Different forms include ichthyosis vulgaris and congenital ichthyosis.
Causes
For almost all types of ichthyosis, a defective carrier of the genetic makeup is the cause. Since defects in genes are generally hereditary, this skin disease is also a hereditary disease. The gene defect causes a disorder in the skin layers. A normal skin formation and exfoliation process is not given. Normally, the skin renews itself continuously. The epidermis is composed of several layers. In its lowest layer new cells are formed, which pass to the layers above. On the way, the cells become cornified and die. Due to cell migration, the horny layer can renew itself about every four weeks. In intact skin, new cell formation and exfoliation are in a healthy balance. In ichthyosis (Ichthyosis), on the other hand, the natural exfoliation is impaired, so that visibly large skin scales develop.
Symptoms, complaints and signs
Depending on its type and severity, ichthyosis can cause quite different symptoms, complaints and signs. Typical of fish scale disease is extensive, plate-like keratinization of the skin. These, usually scaly skin lesions, may be present from birth or develop in the first weeks and months after birth. Ichthyosis vulgaris usually breaks out three to twelve months after birth. This form can be recognized by the gray to greenish skin changes that appear on the extensor sides of the arms and legs. Occasionally, the fine skin scales also appear on the scalp. The affected skin is dry and soft and peels off when touched. The skin color can be brownish to yellowish, occasionally there is bleeding under the cornification, which turns the skin in the affected area reddish. Typical for ichthyosis vulgaris are also ichthyosis hands and feet. In this case, the skin lines take on a rough shape that makes the skin appear wrinkled in some areas. In a quarter of patients, symptoms of neurodermatitis are added. The risk of asthma is also increased. If ichthyosis is treated early, the symptoms can be quickly alleviated. In the absence of treatment, serious respiratory infections and other complications can develop.
Diagnosis and course
Diagnosis of ichthyosis is possible after recording the symptoms and a detailed analysis of the appearance. The type of scaling, the characteristic pattern of infestation, and the accompanying symptoms indicate the particular form of ichthyosis. Depending on the suspected form of the disease, further specific diagnostic methods are then used. For example, the physician analyzes minute tissue samples. Furthermore, a potential enzyme deficiency can be detected in the samples or a damaged genetic carrier can be confirmed by DNA analysis. The course of this skin disease is always protracted. How severe it is depends on the form of the disease. In very severe forms, a sick infant may die shortly after birth. In lighter and moderate forms of ichthyosis, specific complications usually arise in advancing age, due to the dry and rigid skin of the affected person.
Complications
Due to ichthyosis, there are severe discomforts and symptoms on the patient’s skin.However, the symptoms already appear in childhood, so that the diagnosis and treatment of ichthyosis can be carried out at an early stage. As a rule, nodules and scales form on the skin. Both men and women are equally affected by the disease. The skin constantly loses scales, which has a negative effect on the aesthetics of the patient. It is not uncommon, therefore, to experience a decrease in self-esteem and inferiority complexes. However, it is not uncommon for the symptoms of ichthyosis to recede in adulthood, so that there are no further complications or discomforts. It is not possible to treat ichthyosis causally. The affected person can use various creams and ointments to relieve the symptoms. In most cases, baths with various salts also help to limit the symptoms of ichthyosis. Life expectancy is not reduced by this disease. If there are psychological complaints, they can be treated by a psychologist.
When should one go to the doctor?
If typical symptoms such as keratinization of the skin or neurodermatitis signs are noticed, a doctor should be consulted in any case. Ichthyosis can be treated well if it is detected early. People who notice the first warning signs should therefore consult a doctor quickly. This is especially true if risk factors such as neurodermatitis or other skin diseases are present. Babies who show symptoms of ichthyosis shortly after birth must be treated immediately in a specialized clinic. In less severe cases, the family doctor should be consulted. The physician can diagnose the disease and refer the patient to a dermatologist if necessary. If psychological complaints arise as a result of the external changes, a therapist can also be consulted. However, the main focus is on treating the physical symptoms. During the therapy, the skin must be examined regularly and, if necessary, the medication must be adjusted. Therefore, even in the case of mild symptoms, permanent medical monitoring is indicated.
Treatment and therapy
The therapy of ichthyosis aims at alleviating the symptoms. The skin should become more supple through care measures and skin tears should be avoided or treated. This cannot cure the disease, but it can improve the quality of life of the sufferer. Local treatment with specific ointments is an important part of the therapy. Furthermore, in most forms of ichthyosis, the skin should be bathed, rubbed and greased twice daily. Creams containing urea are suitable for this purpose. If the skin is severely affected but not inflamed, vitamin A acid derivatives can also be used. However, these preparations have some strong side effects, so they should only be taken under strict consultation and control with a physician. Apart from local therapy, psychological support for patients often proves useful. Many affected people feel socially excluded by ichthyosis (ichthyosis), so with the help of therapy or a self-help group, these fears can be overcome.
Outlook and prognosis
Ichthyosis is not curable because it is a group of hereditary diseases. However, with good treatment, the symptoms can be greatly alleviated. It should be noted that the skin requires lifelong intensive care. Despite the incurability of the disease, the prognosis of ichthyosis is usually good with appropriate treatment. However, since it is not a uniform disease, in rare cases a severe form of progression may occur. For example, in so-called harlequin ichthyosis, patients often die shortly before or after birth from infections or respiratory diseases. Even in the milder forms of the disease, without therapy and skin care measures, complications can arise from the dry and inelastic skin. Thus, painful tears in the skin are possible. Sometimes sweating is also impeded by the constant formation of calluses. In extreme cases, this leads to circulatory collapse due to overheating of the body (hyperthermia), especially in high summer temperatures or physical exertion. In some cases, neurodermatitis or asthma occur together with ichthyosis.Another consequence of the disease is often the psychological burden on the patients. This can extend to social exclusion and the development of anxiety, which often necessitates psychological care as well.
Prevention
Since ichthyosis is almost always a hereditary disease, there are no special preventive measures. However, if this skin disease exists, it can be slowed down by certain measures. For example, the natural remedies sun and salt water have proven to be beneficial for the diseased skin. Furthermore, those affected should not come into contact with skin-damaging and degreasing substances. These can have a negative effect on the course of ichthyosis.
Aftercare
The aftercare of ichthyosis usually refers to the alleviation of symptoms. The exact measures depend on the individual form of the disease. The main focus is on intensive care of the affected skin. Contact with salt water and sun has been shown to have a healing effect on the clinical picture, which is why doctors often recommend that their patients spend time by the sea. However, increased sweat production is rather counterproductive. Those affected should also stay away from substances that are harmful to the skin. Degreasing substances can also lead to negative consequences. Due to the drying out of the skin caused by the disease, it needs sufficient water and fat. Patients can choose from special gels and lotions for this purpose. The doctor knows which products from the specialist trade are particularly effective here. During a consultation in the doctor’s office, those affected can also find out what an individual treatment with urea, salicylic acid or other active ingredients might look like. Bath oils, soft brushes and sponges are available to rid the skin of cornification and dandruff. Hard areas can be softened in a steam bath. The attending physician will make appropriate recommendations and can also provide information on dietary changes and stress avoidance. If the job is responsible for the condition, sufferers should consider changing.
Here’s what you can do yourself
Ichthyosis must always be treated depending on the individual signs of the disease. Since the skin is dried out, it needs water and fat, preferably in the form of special shower gels and lotions from specialty stores. In consultation with the doctor, ichthyosis can also be treated with salicylic acid, urea, lactic acid or vitamin A acid. Desquamation of the skin works best with table salt and bath oil. Steam baths soften keratinization and allow for gentle removal with a sponge or soft brush. For internal treatment, the doctor’s advice always applies. As a rule, the physician will prescribe acitretin tablets, the effect of which is supported by a change in diet and avoidance of stress. In addition to these measures, affected person should also take preventive measures. In most cases, for example, a change of profession is useful, because the sensitive skin needs a suitable environment in order not to fall ill again. Furthermore, sweat production must be reduced, especially in the summer months. Cooling drinks and a first aid kit should always be at hand. However, should heat stroke or febrile seizure occur, it is best to call the emergency services immediately.
