Ichthyosis autosomal dominant - causes, symptoms, treatment, photos
- 19 Sep 10:15
- Disease Of The Skin
Ichthyosis is a skin defect characterized by a disturbance of normal keratinization. There are several types of disease, all of which are inherited, however, the type of inheritance is different.
Vulgar( second name - normal) ichthyosis occurs more often, thisdisease is transmitted from parents to descendants, the type of inheritance is autosomal dominant.
Ordinary ichthyosis occurs in varying degrees of severity often. According to various data, the incidence of this disease is from 1: 250 to 1: 4000. This disease belongs to a group of disease called atopic dermatitis. About the features of feeding a child with these diseases you can find out by visiting here.
- 1 Causes of development of
- 2 Clinical picture of
- 3 Forms of the disease
- 4 Methods of diagnosis
- 5 Treatment of
- 5.1 Treatment of folk methods
- 6 Forecast and prevention of
- 7 Photo
Causes of development of
The main reason for the development of vulgar ichthyosis is a gene mutation transmittedinherited.
. To this process, the biochemical mechanisms of the process failed to be completely deciphered. However, it was found that skin reactions are provoked by a decrease in the rate of exchange of fats and amino acids. And permanent failures in the exchange of fats in the future may lead to the development of such a disease as lipid necrobiosis. As well as reducing the rate of metabolism in patients in the tissues of the skin, the concentration of enzymes involved in the oxidation processes is greatly increased.
In addition, patients with ihtiosis gradually increase their symptoms, indicating a decrease in the function of the glands of the inner secretion.
Gene mutations in ichthyosis lead to a violation of the absorption of vitamin A in patients with a disturbance in the functioning of the sweat glands, which leads to the intensification of keratinization processes, causes hyperkeratosis.
Clinical picture of
In most patients with usual ichthyosis( hereditary keratosis), the disease begins to manifest even in childhood. However, the first symptoms of the disease may appear much later.
Vulgar ichthyosis may have varying degrees of severity, depending on the degree of damage, secrete several varieties of the disease. In patients, the skin is covered with horny scales, which may have different sizes. The color of the scales may be different, from milky to white to dark gray.
The skin of the patients is very dry and rough to the touch, especially strongly symptoms of the disease are expressed on the knees and the outer bends of the elbows. But the surface of the neck, the skin on the inner surface of the elbow flexion, in the armpits and in the popliteal frames, as a rule, remains smooth.
In vulgar ichthyosis, follicular keratosis is almost always present. In patients in the mouths of hair bags, cornea plugs are formed. In ichthyosis, the skin of the face is rarely affected, however, some adult patients may experience severe peeling of the forehead and cheeks.
In patients with vulgar ichthyosis there is peeling of the skin and the appearance of a characteristic mesh pattern on the soles and palms. The process often involves nails and hair. They become brittle and very dry.
Forms of the disease
Depending on the severity of the symptoms, there are several forms of vulgar ichthyosis.
- Xoderma. This is the shape with the easiest course, has a reversible character. The disease is characterized by dry skin, the appearance of low severity of roughness. As a rule, the most obvious manifestations of this form of ichthyosis can be observed on the knees and elbows.
- Simple Ichthyosis. This form of the disease manifests itself throughout the body. The skin of the limbs, the trunk, the head turns out to be covered with small, densely adjoining to the surface of the scales.
- Brilliant Ichthyosis. For this form of the disease is characterized by the appearance of delicate, translucent scales on the skin, which are located in the form of a mosaic. Most often, the skin of the legs and hands is affected. At an early stage, this disease can be confused with a shiny spiny lobe.
- White Ichthyosis. In this case, the variety of diseases of the scales have a white color. The skin of such patients looks like powdered with flour.
- Snake-like ichthyosis. In patients with this form of disease, the skin is covered with gray-brown scales and looks like a snake's cover.
In modern dermatology, the described forms of the disease are evaluated as vulgar ichthyosis with varying( from I to III degree) severity of symptoms. Subjective sensations in normal ichthyosis, most often, are absent, however, some patients complain of such itching as with itching dermatosis.
Ichthyosis is a chronic pathology that lasts until the end of life, although often the symptoms of the disease are mitigated with age. Aggravation of the disease occurs, as a rule, in the winter, because dry air enhances the symptoms of the disease.
In patients with vulgar ichthyosis, often bad teeth, multiple caries leads to an early loss of teeth. Also, can be observed and candidiasis of the oral cavity. Often, ichthyosis is complicated by eye damage - chronic conjunctivitis, progressive short-sightedness.
In addition, in patients with ihtiosis, immunity is usually reduced, which leads to frequent infectious diseases, susceptibility to allergies. With age in patients with ichthyosis, liver disease and damage to the cardiovascular system often occur.
The basis of the diagnosis of vulgar ichthyosis is a patient's review and anamnesis. It is necessary to find out if there were cases of illness among relatives, and there are no other skin problems in the patient.
Sometimes biopsy and further histological examination are necessary to confirm the previous diagnosis.
The following histological picture is characteristic for ichthyosis:
- Expressed hyperkeratosis;
- Thinning or complete absence of a granular layer in the epidermis.
- Reduced thickness of the studded layer. In the stud-tailed layer you can notice signs of spotted skin atrophy.
- In the basal layer of the epidermis in patients, an increase in the concentration of melanin is sometimes observed.
- Sebaceous glands are atrophied with ichthyosis, and pure hair follicles are reduced.
There is no medicine that could permanently cure ichthyosis. In the treatment of this disease, vitamin therapy is widely used. Patients are prescribed in high doses of nicotinic acid, vitamins of groups B, C, A, E. Vitamin therapy courses should be repeated regularly.
For external treatment, use keratolytic preparations that can soften the scales. The composition of such drugs includes lactic acid, propylene glycol, glycerol.
To support immunity, administration of drugs containing gamma globulin, calcium, iron, aloe extract is prescribed. And how to increase immunity in a disease like herpes, you can learn from this article.
Treatment by folk methods
Folk healers recommend to prepare an ointment based on medicinal herbs to treat ichthyosis. It will be required: 500 ml of finished butter with a St. John's wort( sold in the pharmacy), 50 grams of propolis, 100 grams of beeswax and pine resin( gum), 30 grams of crushed grass, celandine and chalk powder, as well as 250 grams of natural butter.
The ointment is prepared as follows:
- Wax rubbing, mixing with oil in a saucepan at low heat.
- The mixture is heated to a boil, it is added to the resin. Store the boil for 10 minutes.
- The chalk and celandine is poured, the butter is infused with St. John's wort.
- Everything mixes and gets worse with 2 hours of low heat. Next, add propolis and warm up for 30 minutes.
- Let the contents stand 24 hours, then boil again and strain. Used to lubricate the skin during ichthyosis.
Forecast and prevention of
The usual ichthyosis has a lifecycle, so the prognosis for full healing is unfavorable.
Prevention of this disease consists in the passage of medical-genetic counseling before planning pregnancy, if there are cases of ihtiosis in the family of the spouses.