Is ichthyosis transmitted from mother to child. What is ichthyosis and how to get rid of it? Clinical manifestations of ichthyosis

Inherited ichthyosis - combines a wide group of skin diseases that affect mainly keratinized skin.

Reasons for the development of ichthyosis

Pathogen and development conditions this disease To date, it has not been possible to identify, doctors only know that the basis of this disease are violations in the structure of human genes that determine the shape and content of keratins. The main varieties of ichthyosis are the lamellar form of the disease, in which the amount of transglutaminase elements of keratin and proliferative hyperkeratosis decreases, and there is also an ichthyosis of the X-shaped form of the disease, which provokes a sharp deficiency of sterol sulfatase elements.

Symptoms of ichthyosis

On the human skin, traces of exfoliation of the upper layer appear, which resemble the shape of fish scales. This violation of the structure of the skin can have a different degree of threat to health and even human life. Ichthyosis can be manifested by a barely noticeable exfoliation of the upper layer of the skin, and there are varieties of ichthyosis, which leaves very deep wounds on the human body, which are sometimes incompatible with life. Often, along with the development of ichthyosis, the human body falls under the influence of a number of diseases associated with this disease.

Ichthyosis of the usual form

This type of ichthyosis is the most common variant of this disease. Children are predominantly affected by this disease. preschool age, in the period from 1 to 2 years. Scientists have noted the fact that not a single case of illness in children under 3 months of age is known, which is explained by the flow of metabolic processes in the skin system of babies, which take place only in the first months of human life.

Congenital ichthyosis

This form of ichthyosis combines the manifestations of a congenital type of disease and erythroderma. The disease develops as a result of damage to the functional system of the skin, which is responsible for the level of keratinization of the human skin. The disease is transmitted through the hereditary line, autosomal recessive type. A child can inherit this disease if both parents are a carrier of the disease, and then not in all cases, which is associated with the peculiarities of the change in this gene.

As a rule, the disease can manifest itself in several main forms - this is ichthyosis of mild and severe types. The first symptoms of the disease appear even during the prenatal life of the child, but it is not possible to diagnose the onset of the disease during the normal development of the fetus.

Ichthyosis with tinea pilaris

This form of ichthyosis is a hereditary type of disease. The disease has very minor manifestations, quickly manifests itself and is effectively eliminated. The disease belongs to the so-called abortive ailments. In most cases, the disease occurs in children older than the age category.

The disease begins to manifest itself during a person's puberty. Both girls and boys suffer from the disease. The focus of the concentration of lichen is the area of ​​\u200b\u200bthe arms (in the area of ​​\u200b\u200bthe elbow joint), back, hips and buttocks. The manifestation of the disease is small, pointed nodules on the skin, which are fatly located on the skin, forming an interconnected structure. Lichen destroys hair follicles and sweat glands, leaving behind skin scars that often resolve on their own with age.

Treatment of ichthyosis

Ichthyosis acquired with age responds well to treatment and prevention, but many forms of congenital ichthyosis are very difficult to eliminate.

In the treatment of this disease, doctors actively practice techniques for effectively moisturizing the stratum corneum of the skin. To do this, it is recommended to take baths followed by procedures such as applying moisturizing creams and ointments to the skin, which are based on propylene glycol, glycerin, lactic acids and many other keratolytic agents. Moisturizing ointments can be used with or without occlusive dressings.

Prevention of ichthyosis

To date, it is impossible to eliminate the causes of the development of the disease. Parents planning the birth of a child can only prevent the development of pathologies and serious symptoms in their child associated with the action of the ichthyosis pathogen. Parents of unborn children can visit one of the numerous centers in our state where research is being carried out on the processes of change in the chains of the human genome and get expert advice on the possibility of giving birth to absolutely healthy children.

Remember that the care and responsibility for the birth of happy and healthy children lies only with the parents. Decisions about expanding your family with a child should be considered and fully weighed.

A skin disease characterized by thick, dry, and scaly skin. In some forms, patients have intolerance to serious physical activity and intense heat, as the skin loses its ability to sweat.

Unfortunately, ichthyosis can be more than just a cosmetic problem. Sometimes it significantly complicates the normal functioning of the skin. Secondary infections are considered the most common complication. Their occurrence is due to the fact that dry skin is prone to cracking, and favorable conditions arise in the cracks for bacteria to enter and actively multiply.

Hyperkeratosis in ichthyosis is a pathological process in the form of the appearance on the skin of scales resembling fish scales.

Causes

As a rule, ichthyosis is inherited from generation to generation, and it can be noticed in a child at a fairly early age (up to four years), but sometimes it manifests itself immediately at birth. The main reason in this case is a gene mutation.

Sometimes acquired ichthyosis occurs (senile, symptomatic). Its key causes: endocrinopathy (insufficient function of the adrenal glands, gonads, thyroid gland), diseases of the hematopoietic system, hypovitaminosis (lack of various vitamins in the body), senile skin involution (age-related changes).

There are many forms of ichthyosis and a number of rare syndromes.

Symptoms

The symptoms of ichthyosis are best seen in winter period when cold weather sets in with minimal air humidity. With ordinary (vulgar) ichthyosis, which occurs most often, the skin first acquires roughness and dryness, and later becomes covered with gray-black or whitish small scales, firmly connected to each other. At the same time, the popliteal and elbow folds, inguinal region, and armpits remain unaffected. Carriers of the disease often suffer from brittleness and thinning of nails, hair and teeth. As a complication, conjunctivitis may appear. In addition, the disease is accompanied by a decrease in immunity, which, in turn, leads to allergies.

Diagnosis of ichthyosis is based on several factors: the age at which the patient had the first symptoms; personal and family history of ichthyosis; the presence of other skin diseases. Sometimes it is necessary to conduct a series of tests in order to exclude alternative possible reasons scaly and dry skin. One of the most effective ways- skin biopsy.

Treatment

Regardless of what the initial causes of ichthyosis are, it cannot be completely cured. Currently, only symptomatic therapy is carried out. It is prescribed by a dermatologist based on the severity of the course of the disease.

Indispensable components of the pathology elimination course are vitamins of groups A, B, C, E, as well as nicotinic acid. They help soften the scales. Creams and ointments containing lipamide and vitamin U are considered an effective addition to them.

If there is a need to stimulate the immune system, then preparations with a high content of iron and calcium, aloe extract, gamma globulin and blood plasma transfusions are prescribed. Severe stages of the disease, like its congenital form, often require hormonal therapy.

Widespread local treatment ichthyosis. It consists in taking baths with the addition of a solution of potassium permanganate (potassium permanganate). Good results in the fight against the disease bring ultraviolet irradiation, heliotherapy (sunbathing), thalassotherapy (a set of spa treatments based on sea ​​water and mud, algae, silt), carbonic and sulfide baths.

Sincerely,

Ichthyosis of the skin is a hereditary disease that occurs as a result of a violation of the processes of keratinization. In dermatology, several forms are distinguished, which are caused by different groups of defective genes. To understand what kind of disease it is, it is necessary to consider in detail the mechanism of its development. Ichthyosis appears in the form of small light or dark brown plates resembling fish scales. It proceeds according to the type of dermatosis and in case of severe deterioration of the skin condition gives serious complications.

Appearance mechanism

Ichthyosis disease belongs to the category of genodermatoses with a poorly understood mechanism of occurrence. The reason for its development is a gene mutation that is inherited from parents to children. Ichthyosis is a congenital disease, so it is impossible to completely eliminate its manifestations.

Metabolic metabolism failures, when aminocarboxylic acids accumulate in the blood and lipid absorption slows down, are the result of a gene defect. Violation of protein metabolism and an increase in the concentration of cholesterol in the blood serum is the main of the key causes of the appearance of genodermatosis. With a slowdown in biochemical and intensification of oxidative reactions, a violation of thermoregulation occurs. In most patients suffering from genodermatoses, dysfunction of the adrenal glands, thymus gland and gonads (ovaries and testicles) is diagnosed.

Congenital ichthyosis is genetically transmitted and is not contagious. In connection with the violation of the absorption of retinol, there are malfunctions in the sweat and sebaceous glands. Therefore, the processes of keratinization of the epidermis go wrong: the thickening of its stratum corneum is excessive (hyperkeratosis). Excessive synthesis of keratin leads to a change in the structure of the skin and a slower rejection of dead cells. Keratinocytes increase the density of the skin, and therefore a characteristic pattern appears on the body, resembling fish scales.

The function of the epidermis is to protect the deep layers of the dermis from pathogenic fungi, protozoa, bacteria and viruses. But if it is damaged by ichthyosis, resistance to pathogens decreases, which creates conditions for infectious tissue inflammation. In adults, the disease is sometimes complicated by pathologies of the liver, heart and other internal organs.

Symptoms and causes of different types of ichthyosis

Signs of ichthyosis appear from the first months of life in the form of excessive peeling, dehydration and thickening of the skin. Organic acids accumulate between the scales, which have pronounced cementing properties. Therefore, the skin of patients becomes rough and rough to the touch. The severity of clinical manifestations of genodermatosis varies greatly. A mild form of ichthyosis practically does not cause psychological or physical discomfort to patients.

In dermatological practice, 28 forms of the disease are distinguished. Each of them is characterized by its own symptoms and mechanisms of violation of the processes of keratinization of the epidermis. The most commonly diagnosed types of ichthyosis are:

• Vulgar. It occurs in most patients suffering from ichthyosis-like diseases. Ichthyosis vulgaris manifests itself in the first few years of life. Genetic failures and defects that are transmitted in an autosomal dominant manner are the cause of its development. The disease is disseminated in nature - it affects almost the entire body. Most often, lesions are localized on the extensor surfaces lower extremities. Translucent scales form on the skin, which often clog the mouths of the follicles. Distinctive feature ichthyosis vulgaris is the absence of cornification areas in the flexion zones - under the knees, in the armpits, etc.
• Lamellar. This genodermatosis develops due to a defect in transglutaminase, which regulates differentiation, that is, the future "profile" of epidermal cells. Mutations in protein components lead to accelerated growth of the basal layer, causing the skin to thicken. Lamellar ichthyosis in newborns is manifested by the formation of a light brown film on the body. Over time, it takes the form of large scales. In patients, the skin on the face looks reddened and stretched. In some areas, there is excessive sweat and sebum secretion.
• X-linked. This form of the disease manifests itself only in boys, girls are carriers of mutated genes. Genodermatosis leads to insufficient production of steroid sulfatase, which is involved in the transformation of steroid hormones. Symptoms of the disease appear within 2-3 weeks after birth. Small plates resembling scales form on the skin. Such patients are often diagnosed with mental retardation and abnormal development of the skull.
• Follicular dyskeratosis. The disease affects not only the skin, but also the thymus and sex glands. It begins due to a defect in the enzymes involved in cell keratinization. It is characterized by the growth of the outer layer of the epidermis, a violation of keratinization and manifests itself in the form of nodular rashes on the skin. Over time, the scaly papules coalesce, resulting in weeping lesions.
• Ichthyosiform erythroderma. Autosomal dominant pathology occurs due to the production of defective keratin. The process of keratinization is disturbed due to changes in the differentiation of epidermal cells. Small bubbles form in the outer layers of the skin, and a thickening of the stratum corneum is also observed. It is manifested by soft tissue edema, weeping foci, delamination and thickening of the nails.

Ichthyosis is often accompanied by disturbances in the work of the endocrine, musculoskeletal and nervous system. Most often, the adrenal glands, gonads and thymus are affected. The main manifestations of the disease include dryness, peeling, hyperemia (redness) and deformation of the nail plates.

How dangerous is this

Mild genodermatoses do not cause much discomfort to patients, but in some cases ichthyosis can lead to severe complications. The disease is difficult to treat and rarely regresses. It proceeds in waves with periods of exacerbation and remission. Symptoms are aggravated in the cold season, causing the following complications:

• retinitis (inflammation of the retina);
• hormonal disorders;
• secondary infections;
• purulent inflammation of the skin;
• blood poisoning;
• chronic conjunctivitis.

Congenital ichthyosis of the skin in children leads to dysfunction of vital organs, which causes death. The prognosis worsens with endocrine and cardiovascular diseases.

Features of diagnostics

During intrauterine development, it is difficult to diagnose this anomaly. To reduce the risk of having a child with ichthyosis, they resort to molecular genetic diagnostics. With a high probability of gene mutation leading to genodermatosis, doctors recommend refraining from pregnancy.

It is easy to determine ichthyosis in children and adults by the characteristic clinical manifestations. Congenital forms of the disease are differentiated from scaly lichen, erythroderma, neurodermatitis. To confirm the diagnosis, the following studies are carried out:

• histological analysis;
• clinical blood test;
• microscopic examination.

To detect skin diseases in early pregnancy, they resort to a biopsy of fetal tissues, which is carried out at 20-21 weeks. In this case ultrasound examination not considered informative.

How to treat ichthyosis

Methods of therapy are determined by the characteristics of the course of genodermatosis. Treatment of skin ichthyosis involves the use of drugs from several groups, the action of which is aimed at restoring the processes of exfoliation of keratinized cells and softening the epidermis. To relieve the symptoms of the disease, apply:

• keratolytic agents (silver nitrate, Ichthyol, Naftalan) - eliminate hyperemia, relieve inflammation and accelerate the exfoliation of skin cells;
• retinoids (Adapalen, Roaccutane, Airol) - compensate for the lack of vitamin A in the body, which contributes to the normalization of skin functions;
• hormonal ointments (Kremgen, Akriderm, Diprosalik) - eliminate inflammatory processes in the skin and prevent the occurrence of ulcers;
• membrane-protective agents (Dermatol, Videstim, Retinol-gel) - accelerate tissue regeneration, moisturize the skin and reduce the likelihood of inflammation.

Local treatment consists in taking starch, salt and herbal baths. To relieve inflammation, use decoctions with tansy flowers, medicinal chamomile and calendula. To eliminate the symptoms of ichthyosis, physiotherapeutic procedures are used - thalassotherapy, UV irradiation, mud therapy, heliotherapy.

The intake of fortified products is an integral part of the treatment of genodermatosis. For partial restoration of skin functions and acceleration of metabolic processes in it, water- and fat-soluble dietary supplements are taken. With ichthyosis, it is recommended to use drugs that contain retinol, pyridoxine, thiamine, tocopherol, ascorbic acid and cyanocobalamin.

Skin care and diet

The disease is often complicated by secondary infections that enter the body through cracks on the surface of the epidermis. Treatment of ichthyosis at home involves following certain rules for caring for dry skin. To prevent complications, you must:

• regularly use moisturizing ointments and body oils;
• treat damage with antiseptics;
• take baths with herbal decoctions, potassium permanganate;
• apply keratolytic ointments to the lesions;
• make applications with wound healing liniments.

Proper nutrition with ichthyosis has a beneficial effect on the condition of the skin and the well-being of patients. To restore the biochemical processes in the body, the diet includes foods high in vitamins:

• dried fruits;
• oatmeal;
• radish;
• tomatoes;
• garlic;
• sour cream;
• cottage cheese;
• seaweed;
• nuts;
• buckwheat;
• sea ​​buckthorn;
• currant.

To prevent complications, it is advisable to exclude from the menu products that adversely affect the functioning of the gastrointestinal tract:

• confectionery;
• semi-finished products;
• carbonated drinks;
• alcohol;
• spicy food.

In the spring-autumn period, it is advisable to take dietary supplements that contain trace elements, vitamins of groups B, C and A.

How to cure ichthyosis forever

The genetic disease ichthyosis cannot be completely cured. So far, scientists have not developed etiotropic drugs that could eliminate mutations in genes. Skin treatment is carried out with symptomatic medications that reduce the severity of clinical manifestations. Ichthyosis is characterized by an undulating course, so at best a stable remission can be achieved.

To prevent exacerbation of genodermatosis, you should:

• increase the humidity in the room with humidifiers;

Under the diagnosis of "ichthyosis" various diseases can be hidden. This is a group of skin lesions that have similar clinical manifestations.

Ichthyosis

What is ichthyosis? The name of the disease comes from the Greek word for "fish", as the skin in such patients resemble scales. Usually they appearance very specific, and the diagnosis is established during the first examination by a dermatologist.

However, there are different forms and severity of the disease, on which the severity of clinical symptoms depends.

The most common types of ichthyosis are:

• Ordinary, or vulgar.
• Lamellar.
• Linked to the X chromosome.
• Broca's ichthyosiform erythroderma.
• Congenital.
• Hair lichen.

The reasons for the development of ichthyosis are gene breakdowns that are inherited. Spontaneous mutations are also possible.

Ichthyosis common (vulgar)

This is the most common form of ichthyosis. She debuts at the age of 1–2 years. In rare cases, symptoms appear earlier, but they never occur before the age of three months.

The maximum skin manifestations are noted at the age of 8–10 years, after which they persist throughout life. Improvement usually occurs during puberty and in the summer.

For ordinary ichthyosis, the following symptoms are characteristic:

• Dry skin.
• Peeling, which is most pronounced on the lateral surfaces of the body.
• Involvement in the pathological process of the forehead and cheeks with the appearance of gray scales on them.
• Absence of inflammatory changes in the skin, its redness.

Throughout the body of a child with ichthyosis, horny scales are noted. At 4-5 years old, they are localized on the face, but then the skin in this area is cleared. On the trunk, the greatest changes in the skin can be seen in the area of ​​the sacrum, ankles, extensor surfaces of the arms and legs.

Scales can be white, thin. In this form, they resemble bran. Some children have thick and dark scales. The armpits, inguinal and intergluteal folds remain intact.

Keratoderma (keratinization of the skin) is noticeable on the palms, soles and perineum.

Depending on the severity of skin changes, a number of forms of common ichthyosis are distinguished.

Forms of ordinary (vulgar) ichthyosis

In dermatology manuals, you can find the following classification of ichthyosis vulgaris:

• Xeroderma.
• Simple ichthyosis.
• Pearl.
• Serpentine.
• Spiny.

The name of the form depends on the type of scales. With xeroderma, they are barely noticeable, similar to bran. This is the easiest version of ichthyosis vulgaris. With an increase in their number and compaction, the skin acquires a pearly sheen. If the scales become even more dense and rough, they begin to resemble the skin of a snake. The most severe form is spiny ichthyosis, when the skin becomes like small porcupine quills. In old age, the scales darken and the patient is diagnosed with Ichthyosis nigricans.

In mild forms of the disease, the hair does not suffer, but in moderate and severe forms, it becomes thinner, brittle and dry. Stops the production of sweat and sebum. Children with ichthyosis are often asthenic, lag behind in psychomotor development. They are prone to frequent colds, otitis, pneumonia, pyoderma.

Ichthyosis vulgaris is an autosomal dominant pathology. This means that it can be transmitted from either parent.

The risk of having a sick child ranges from 75 to 100% if both mom and dad are sick. If ichthyosis is observed in one person, the probability of inheriting the disease is 50%.

Lamellar ichthyosis

This disease is transmitted in an autosomal recessive manner. If both parents are carriers of this gene, in 25% of cases a sick child is born. If one of them is sick, all children will be carriers. Diagnosis can be made immediately after birth.

The child is born with characteristic symptoms ichthyosis:

• erythroderma (redness and swelling of the skin);
• yellow-brown film covering the skin;
• twisted lips and eyelids.

A few days later, there is abundant peeling, which leads to skin cleansing. With a mild form of the disease, the skin can be completely cleared, but this rarely happens. Eversion of the lips and eyelids persists throughout life.

In middle and old age, erythroderma is less pronounced, but hyperkeratosis is clearly visible. Lamellar ichthyosis is characterized by a combination with dementia (dementia).

X-linked ichthyosis

This disease can only be transmitted in a recessive manner. The carriers of the defective gene are women, and the clinical manifestations of ichthyosis are observed in men. In rare cases, girls can also get sick. This is possible in a situation where the father is diagnosed with this form of ichthyosis, and the mother is a carrier of the gene.

With this pathology, the skin is very dry and flaky. Usually the whole torso and face are affected, as well as atypical places - armpits, pits of the elbows, folds in the groin. With X-linked ichthyosis, foci of alopecia are often found. Scales with this ichthyosis are usually dark, dense, large.

Patients often have dementia, eye damage in the form of cataracts, anomalies of the musculoskeletal system, and connective tissue dysplasia.

Broca's congenital ichthyosiform erythroderma

There are two forms of this disease - dry, or non-bullous, and bullous. The dry type of ichthyosis is inherited recessively, and the bullous type is dominant. The pathology gene is not linked to sex, both women and men suffer.

The dry form of Broca's erythroderma is characterized by:

• skin tension;
• hyperemia;
• dryness;
• profuse peeling;
• a large number of scales on the soles and palms, in the natural folds of the body.

With this disease, there are no blisters (bulls), the scales are brown and dense, they are separated from the skin in whole layers. On the soles and palms, keratoderma is determined, in the folds - warty growths.

The disease can maintain its clinical manifestations throughout life. But more often erythroderma is replaced by severe keratosis, most distinct in the area of ​​the body folds. Over time, ectropion (eversion of the eyelids) may form.

Broca's erythroderma is often accompanied by the appearance of foci of leukoplakia, deformation of the palate, upper jaw, lips, subungual hyperkeratosis.

With a bullous form in a child, flabby blisters appear on hyperemic skin, which open on their own. Erosions form in their place. These foci can become secondarily infected, which leads to the development of bacterial complications.

By the age of five, hyperkeratosis increases, and there are fewer bubbles. Rashes are usually located on the back of the hands and feet, neck, large folds of the body. In the bullous form, the scales are dark, have a linear shape, and are difficult to separate from the skin.

Congenital ichthyosis in children

This hereditary disease debuts in utero, it happens in two forms - mild and severe. The type of transmission is autosomal recessive, the degree of manifestation of the signs of the disease is variable.

The severe form is characterized by damage to the child's skin in the form of the formation of a rough shell covering the entire body. There are clearly visible bleeding cracks on it. In medicine, such a pathology is called the "fruit of Harlequin".

Also, the newborn has deformities of the auricles and lips, eyelids, face, limbs.

Horny layers are located not only on the skin, they fill the cavities of the ears, nose and mouth. Congenital ichthyosis of the skin in children is often combined with defects in other organs and systems. With this disease, the child's skin breathing is disturbed, the integument does not fulfill its barrier and protective function. Secondary bacterial complications often join. These are the most common causes of death in babies.

In some cases, congenital ichthyosis regresses on its own. It can also transform into ichthyosiform erythroderma.

The mild form is characterized by severe hyperemia of the skin, keratoderma of the soles and feet, deformity of the ears, eversion of the eyelids. The prognosis is favorable, but the disease requires intensive treatment from the first days of life.

tinea versicolor

Dermatologists classify hair lichen as an abortive variant of ichthyosis. The debut of the disease occurs in childhood, but obvious signs of the disease are found only at a young age. Males and females are equally often affected.

A characteristic symptom of hair loss are pointed nodules, which are located on the limbs, buttocks, back. At the top of the nodule, layers of scales are noticeable, they close the mouth of the follicle. Hair growth and sebum production at the site of the rash stops. In the future, scars of small sizes are formed there.

Diagnostics

Diagnosis is based on characteristic clinical picture- dryness and peeling of the skin, violation of the separation of sebum and sweat. Ichthyosis must be differentiated from the following diseases:

• bullous epidermolysis of newborns;
• congenital syphilis;
• staphylococcal skin lesions;
• xeroderma;
• desquamative erythroderma;
• spinular lichen.

Differential diagnosis is carried out by a dermatologist using special tests and techniques. Each disease is characterized by a set of typical signs, which allows you to confirm or refute the alleged pathology.

The appearance of cases of congenital ichthyosis in the family requires prenatal diagnosis to calculate the risks of having a sick child.

Treatment

The type of ichthyosis and the severity of the disease determine its treatment. In congenital pathology, timely (no later than 10 days of life) hormone therapy - glucocorticosteroids - is important. Usually dermatologists use drugs based on prednisone. An intensive course lasts at least a month, then the dose of the drug is slowly reduced.

In mild forms of ichthyosis, it is advisable to use topical retinoids. These are structural analogues of vitamin A, or retinol, which are applied to the skin. With severe damage to the integument, systemic retinoids are used, but their use is accompanied by a number of side effects.

To eliminate dry skin, doctors prescribe emollients and fattening agents - externally. Aquatherapy is widely used - various baths in which you can add milk or butter, soda, starch, saline solutions. Hot water is not recommended, as it increases the dryness of the skin, contributes to damage to the integument.

With ichthyosis, sea bathing is recommended. A good effect is observed with sanatorium treatment, climate change to warm and humid. Living in areas with increased dryness of the air, wind, and the predominance of cold weather can adversely affect the condition of the skin.

Ichthyosis is a serious disease. It cannot be completely cured, but if the doctor's recommendations are followed, remission and a decrease in clinical manifestations can be achieved.

Ichthyosis is not contagious, its forms are inherited. Symptoms range from mild to severe. Children with this pathology can attend kindergarten and school, children's groups. But they require lifelong follow-up with a dermatologist and treatment if necessary.

Ichthyosis is hereditary disease skin, which proceeds according to the type of dermatosis. A manifestation of ichthyosis are scales on the skin that resemble fish scales. The main cause of ichthyosis is a gene mutation, but the biochemistry that is inherited has not yet been deciphered. The most common form of ichthyosis is ichthyosis vulgaris, which occurs before the age of three. With the development of ichthyosis, other organs are affected.

Clinical manifestations of ichthyosis. Damage to organs and systems in ichthyosis

At first, the skin becomes dry and rough, then whitish or gray-black scales closely adjacent to each other appear on it. With ichthyosis, the areas of the popliteal fossae, elbow bends, the area of ​​\u200b\u200bthe armpits, and the inguinal zone are not affected.

The skin on the palms peels off, and the skin pattern becomes more pronounced. The severity of the course of this skin disease depends on how deep the gene mutation is. In the case of an abortive course, the only manifestation of ichthyosis will be dryness and slight peeling of the skin on the extensor surfaces.

With ichthyosis of the skin, other organs are also affected:

• Nails exfoliate and break off easily, hair becomes dry and brittle, multiple caries appears.
• Often this disease can be accompanied by eye damage - retinitis and chronic conjunctivitis.
• Patients diagnosed with ichthyosis are hereditarily predisposed to myopia, which begins to manifest itself in childhood.
• Due to reduced immunity, patients are constantly faced with allergic diseases and purulent infections.
• Later, problems with internal organs- liver diseases, cardiovascular insufficiency and others.

Features of the development of recessive ichthyosis. Forecast for life

Recessive ichthyosis affects only men. A two-week-old baby has clinical manifestations of the disease: horny skin layers in the form of large dense black-brown scales. The areas of skin between the scales are covered with cracks, for this reason, the skin looks like snake or crocodile skin. In addition, epilepsy is quite common among children with recessive ichthyosis, mental retardation and anomalies in the structure of the skeleton.

The development of congenital ichthyosis occurs at 4-5 months of intrauterine development. The baby is born with skin covered with thick, gray-black horny scutes.

There is a possibility that a child with such a disease will be born with a congenital anomaly of the skeleton - clubfoot or clubhand.

Due to the presence of abnormalities incompatible with life, most children with a congenital form of ichthyosis live only a couple of days.

The main methods of treatment and prevention of ichthyosis

Dermatologist prescribes vitamins of groups A, E, B, C and nicotinic acid in large doses. Preparations containing lipamide and vitamin U are prescribed, which have a lipotropic effect and soften scales.

In order to stimulate the immune system, blood plasma transfusions, gamma globulin, as well as preparations containing iron and calcium, aloe extract are used for ichthyosis.

Hormone therapy for ichthyosis is prescribed if the case is quite severe, or with congenital ichthyosis. In the presence of eversion of the eyelids, an oily solution of retinol acetate is instilled into the eyes. After the condition is normalized, the dose hormonal drugs slowly decreases until complete abolition. Blood tests should be carried out during remissions in order to monitor the patient's condition and prevent the development of complications. A nursing mother should take vitamins similar to those taken by a child with ichthyosis.

With local therapy, patients are prescribed general baths containing a solution of potassium permanganate, as well as the use of a baby cream, to which vitamin A is added. If the patient is an adult, he is shown starch and salt, local and general baths.

The main prevention of ichthyosis is pre-pregnancy counseling in order to determine the degree of genetic risk. If, after an analysis of the amniotic fluid, ichthyosis is detected in the fetus, it is recommended to terminate the pregnancy.