Ichthyosis of the skin in children: Is this disease dangerous for newborns, what to do and how to treat? Skin ichthyosis in children - photos with description, treatment, causes and symptoms Strengthening the immune system
The ichthyosis is a skin disease, in which a person has a violated the process of the epidermis oroging process, as a result of which solid flakes appear on it. In this case, pathologically modified keratin accumulates in the skin of patients.
The disease received its name from Greek "ichthyo", which means fish. This is due to the fact that the skin of patients looks like fish.
Ichthyosis is inherited. In more rare cases, a person may have acquired ichthyosis. The disease is not a consequence of infection, which means that it is impossible to infected.
The first signs of ichthyosis in patients appear in childhood, and sometimes even immediately after birth.
If the disease is caused by genetic disorders, then patients usually have disorders in thermoregulation, slow metabolism is noted. Due to the fact that in the patients in the body in the oxidative reactions there is an increased level of enzymes, they are significantly enhanced by their skin breathing. People suffering from hereditary ichthyosis often suffer from the diseases of the thyroid gland, reproductive organs, as well as adrenal glands. Often they have a deficiency of immunity (cellular and humoral together).
These patients have almost always violated the work of sweat glands, which, in combination with vitamin A deficiency, can lead to an enhanced delicate of skin.
With this pathology in the skin, an excess amount of keratin is always present, the structure of which is broken. At the same time, the rejection of old skin cells occurs extremely slow. As a result of this, scales appear on the human skin, in the space between which amino acid complexes are collected, provoking their hardening. For the same reason, the scales are firmly connected to each other.
In patients suffering from ichthyosis, there are dry and brittle hair and nails, many teeth are amazed by caries.
During ichthyosis, eye diseases are often observed: such as retinit, conjunctivitis and myopia.
Causes of ichthyosis
The main cause of the occurrence of ichthyosis is to mutate in genes, which is accompanied by a violation of the exchange of proteins and fats in the body. In this case, the biochemistry of these processes has not yet been finally studied.
If the ichthyosis is acquired pathology, the reasons for the occurrence of the following adverse factors may be included:
- failures in the work of the thyroid gland;
- violations in the work of the sex glands;
- deficiency of vitamins, micro- and macroelements;
- the pathology of the adrenal glands, which caused the emergence of hormonal disorders;
- serious pathology of the cardiovascular system;
- various changes occurring with leather as aging.
Varieties of ichthyosis
To date, scientists allocate the following types of ichthyosis:
- vulgar ichthyosis or ichthyosis ordinary is the most common form of pathology. It is transferred from parents to children. In a light form, the disease is manifested in pathological dryness of the skin and the formation of gray or white plaques on them. In more severe cases, these plaques become very dense. At the same time, the curves remain healthy on the bends or in natural folds. In patients with this form there are always diseases of nails and hair. It is worth noting that the course of the disease is complicated in the summer, especially in the rainy season. At the same time, in winter, the symptoms of the disease decrease or even can completely disappear;
- abortive ichthyosis is ichthyosis in a light form. With it, the defeat is not all over the body, but only on buttocks and legs;
- black, shiny and white ichthyosis. Distinctive features of black ichthyosis is the appearance of dark brown, almost black color, white - white, and brilliant - shiny;
- X-linked ichthyosis is a pathology due to congenital deficit in the organism of steroid sulfate (substance with which steroids become active). This disease occurs approximately 1 person from 2-6 thousand;
- rapey ichthyosis - from other species of this pathology, is distinguished by the presence of a huge number of horny layers, which have kind of needles or spikes;
- freewood or simple ichthyosis - a distinctive feature of this form of ichthyosis is the presence of a large number of gray shades scales, which are attached to the skin cover of the central region;
- hytiosis Harlequin is the most severe form of the disease. With it, the leather of babies from the moment the appearance of the light is already covered with thick and rude plates. At the same time, deep cracks and splits are already present on their skin. The mass of the plates with a given variety of the disease can be so big that it can pull the skin on the face with its weight and thus distort the features of the face, worsen breathing and make food intimidable. Such children from birth need long and heavy treatment. According to statistics, the harlequin ichthyosis is found approximately 1 of 500 thousand newborn children;
- plate ichthyosis (lamellar). For the development of this pathology, the recessive gene is responsible. The disease occurs equally often among boys and girls, can affect the skin of people, regardless of their race. According to statistics, the lamellar ichthyosis occurs in 1 child of 200 thousand. The disease does not affect the life expectancy;
- recessive ichthyosis. This congenital form of the disease, which boys are faced. In children, patients with recessive ichthyosis, more often than others are diagnosed with a lag in mental development. In addition, they often have disorders in the structure of the skeleton, epileptic seizures may arise. In some cases, this form of ichthyosis is combined with hypogonadism.
Heredity
Women who wanted to become pregnant should know that ichthyosis has an autoimmune dominant character of inheritance.
It means:
- if, in a pair, both parents are carriers of the gene caused by ichthyosis, but at the same time they themselves do not hurt, then 25% is the likelihood that their children will be sick with ichthyosis, 25% do not get sick, 50% - do not get sick, but at the same time they are carriers of the gene;
- if in a pair, one parent suffers from ichthyosis, while the other is not, but is the carrier of the gene causing disease, then the likelihood that children will suffer from ichthyosis, equals 50:50;
- if the mother and father of the child are sick by ichthyosis, their children will also have this pathology;
- if one parent is a carrier of a gene, and the other is completely healthy, then the probability of the birth of a sick child is very small.
It should be noted that the birth of children patients with ichthyosis is significantly rising in pairs that have a blood connection. If a healthy child was born in healthy parents, but over time he had this pathology, it may indicate that a spontaneous mutation of a recessive gene occurred in the body's body.
Effects
Congenital ichthyosis refers to the most unpleasant forms of the disease. In such patients, skin covers are completely covered with solid plates. Because of this, their skin becomes like a shell. After some time, the plaques on the skin acquire a grayish yellow color.
In this case, patients may be present in the twist of the eyelids. Children who have congenital ichthyosis are usually born before the deadline, so they have problems with breathing organs from birth.
Many people who are ill with ichthyosis are mentally unhealthy.
because of weak immunity They increase the risk of infection and development of respiratory failure, as well as liver diseases.
That is why the treatment of ichthyosis must be done necessarily. The therapy of this pathology is engaged in a dermatologist. However, in the presence of concomitant diseases, the help of an immunologist, a nutritionist, a neuropathologist, a psychotherapist, an oculist, etc. may also need.
Only a doctor will tell patients, how to wash with ichthyosis and how to properly care for the body. It will appoint special creams, ointments and medication, which will greatly facilitate the life of the patient.
The forecast of ichthyosis is unfavorable, since exchange disorders accompanying pathology, sooner or later will cause complications and exacerbate the course of the disease.
The prophylaxis of ichthyosis consists in careful observation during pregnancy. So, when the amniotic fluid is detected in the analysis, the doctors recommend interrupting pregnancy.
Params that the risk of child's gear is very high, it is recommended to adopt children.
Symptoms
Such a skin disease like ichthyosis has its own signs, seeing which should immediately apply for medical help and subsequent treatment to a dermatologist. It will conduct an outdoor skin inspection, then prescribes diagnostics using tests and would be effective treatment.
The manifestation of any symptoms of ichthyosis depends on the type of disease, as each type has its own characteristics and treatment schemes.
Symptoms depending on the type of disease
Most often, this disease is manifested in the first months of the child's life, or in a later childhood. Ichthyosis, the signs of which differ in the nature of the appearance, duration of distribution, can be dangerous to the health of the child, and in some cases - for his life.
Symptoms of ichthyosis can be the following:
Vulgar ichthyosis
The dermatologist can diagnose it in some cases by 4 months of life:
- at the stage stage, the skin of the baby becomes dry, and on the touch of a rough;
- in the 2nd stage, it begins to be covered with flakes that have a whitish color and firmly fit to each other. The main zones of the lesion is the skin of the hands and feet, and the armpits of the depressions, the elbow bends and the patellied pits are not subject to the appearance of scales;
- pathological changes affect not only skin covers, but also hair, nails, teeth. Their condition deteriorates: the hair becomes brittle, nails peel off, and caries appears on the teeth;
- due to the decline in immunity, various infections are negative to the body. Such children often experience problems in the functioning of cardiovascular and respiratory systems.
Congenital ichthyosis
You can diagnose immediately at the birth of a child:
- the baby's skin is usually covered with powerful horny layers that resemble the skin of the crocodile;
- at birth, the skin covered with a film of yellowish color is covered, then it peckles, after which there is a strong redness of the skin throughout the body;
- for severe course diseases, it is possible to form bubbles on the skin;
- in addition to the lesion of the skin may be discovered by the disease of the endocrine system, central nervous system, dystrophy nails and hair, as well as eye damage.
Acquired ichthyosis
Could be diagnosed due to malignant tumors, metabolic disorders, long-term reception of certain medicines. The following signs are present:
- dryness and peeling of the skin;
- hypovitaminosis;
- pathological processes in the kidneys.
Fetal ichthyosis (Harlequin syndrome)
It occurs even during the embryonic development of the fetus (about 4 months), has the following symptoms At birth:
- severe dry skin, it looks like a shell with large scales;
- eyelids turned outward;
- deformation of parts of the face;
- incorrect and disproportionate structure of hands and legs.
Most often, such children are already born dead, either die in the first months of life.
Epidermolithic ichthyosis - skin covers are bright red;
- it is characteristic of the formation of bubbles on the skin, which have the burst property, after which the wound surface is formed.
When attaching an infection can occur the abscess and death of the child.
Ichthyosis, whose symptoms can manifest themselves at any age, is considered a disease that is not treated in full, it can only be supported during periods of exacerbation. With all types of ichthyosis, the patient suffers to the disorder of the functions of sweat glands, the defeats of the body's immune system, slowing the metabolism.
Diagnostics
Ichthyosis is a dermatological disease characterized by the formation of dry scales on the skin similar to fish or reptiles. For this feature, the disease and got its name. The disease is usually caused by genetic hereditary failure. Ichthyosis can develop in a child in the womb or diagnosed later.
The diagnosis of ichthyosis usually occurs by an outdoor examination of the patient. When examining, the doctor should find out the following points:
- The age at which the first symptoms of the disease were manifested;
- Did someone their members of the family ichthyosis sick;
- What other skin diseases are present in the patient's history.
Answers to these questions will simplify the diagnosis and help to determine the etiology of the disease in each case. Since there are various forms of ichthyosis, it is important when diagnosing it is important not to simply identify the disease, but also its form of manifestation. It is necessary in order to correctly choose treatment.
The diagnosis of ichthyosis should be engaged in a dermatologist. He is external signs Will be diagnosed, and in some cases of these signs, it is enough to distinguish ichthyosis from other similar diseases. In external examination, the doctor draws attention to the manifested signs of ichthyosis. They can be different depending on the form of the disease. The most common form is vulgar ichthyosis. He is manifested in early childhood no later than a three-year-old. For this disease, it is characterized by education on the skin of small-sized white or light gray scraps. Scales can affect the whole body or small areas. As a rule, the groin area remains intact, the armpits, bends of elbows and knees remain untouched.
Over time, scales can energize and acquire a brown tint. This usually happens in the absence of timely and effective treatment. In addition, with vulgar ichthyosis, sweating significantly decreases due to the fact that sweat glands have insufficient development. Hair and nails usually have a deformed state. This is due to a disorder in the synthesis of keratin, from which the structure of hair and nails are for most part.
There is also a rowed form of congenital ichthyosis, in which the newborn is intensively distinguished by the skin secretion, which then dries out. Due to this, a dense yellowish crust is formed on the skin. This type of ichthyosis (in contrast to the previous one) is healing, but during the diagnosis can cause some difficulties. Since the crust does not always acquire a scaly shape characteristic of classical ichthyosis.
But the lamellar congenital ichthyosis during the diagnosis of difficulties does not cause. With such a variety of disease, the child is born with a film that covers its skin. After some time, the film disappears, and under it there are scales characteristic of ichthyosis. Unlike the previous one, this form is not curable, but is transferred easier than vulgar ichthyosis.
The boys meet a recessive form of ichthyosis. In this case, shallow cracks are formed between the scales. The skin is reminded by the skin of the snake or crocodile. The scales themselves are very solid and dense, have a dark shade. Very often, this disease is accompanied by such diseases as epilepsy, Down syndrome, cataract, underdevelopment of the musculoskeletal system.
In rare cases, patients appeal to the doctor with the acquired form of ichthyosis. It arises against the background of various violations of hormonal background or work gastrointestinal tract. If, as a result of the diagnosis, it turned out that the ichthyosis is acquired, then additional surveys should be prescribed to eliminate the presence of tumors having a malignant form. Acquired ichthyosis is often preceded by leukemia, sarcoma or tumors of various organs. Most often, the organs of the urogenital system are suffering, so in secondary diagnostics should pay special attention to them.
In addition to the condition of the skin when diagnosing, the doctor draws attention to the teeth. Very often, patients suffering from ichthyosis have caries on teeth. At the same time, fragile sparkling nails and thin hair can help confirm this assumption.
Also, during the diagnosis, you should pay attention to the state of the eyes. Various forms of conjunctivitis and the rapid development of myopia are accompanying diseases during ichthyosis.
With outdoor inspection, you should pay attention to the feet and palm. The drawing on them is usually more pronounced. And the folds can be amazed dry and peeling. Sometimes it seems that white flour in small quantities gathered in the folds.
Forms of manifestation of ichthyosis can resemble such diseases as:
- Palm and plantar keratosis - thickening of the layers of the skin in the field of palms and feet, in the area of \u200b\u200bthe defeat and the type of destruction, you can visually distinguish between ichthyosis;
- Red hair deprived - the skin first covers watery papules, which hike resemble scales during ichthyosis. When diagnosing, it is necessary to find out whether there were such papulas, accompanied by redness, itchy and increased temperature Body.
In addition to outdoor inspection, if necessary differential diagnosis And the exceptions of other dermatological diseases are prescribed a biopsy of the affected area and histological examination. Biopsy can be prescribed during pregnancy, it will show that the skin of the fetus is abnormally thickened. This will be evidenced by the fact that with a high probability, the child will be born with ichthyosis.
So, the clinical picture of the disease helps the doctors of dermatologists to easily determine such a disease as ichthyosis. It is quite easily diagnosed, but has the likelihood of both other passing diseases. Most often ichthyosis is observed at an early age or immediately after the birth of a child. But there are cases when ichthyosis is purchased throughout life and manifests itself after 20 years against the background of gastrointestinal disorders or hormonal pathologies.
Treatment
Ichthyosis is a genetic disease, therefore, answering the question "can Ichtiosis be cured?" Doctors argue that this skin disease is completely incurable. Therapeutic process is directed mainly to alleviating the condition of the patient, eliminating adverse symptoms arising from the exacerbation of the disease. When the very first symptoms are found, it is necessary to immediately refer to the dermatologist, which will conduct diagnostics and prescribe the appropriate treatment.
Ichthyosis is treated in different ways: both drug and folk remedies. If you decide to begin treatment of ichthyosis with the help of people's ways, it is necessary to take into account that you first need a doctor consultation, which will explain whether these treatment methods can be used in relation to this disease at home.
Medical method of treatment of ichthyosis
In order for the effectiveness of therapeutic process to be high, the duration of exacerbation is as short as possible, and the relief of symptoms passed by an operational, the dermatologist can be appointed the following drugs and ointments:
- Vitamins - groups C, B, A, E, PP (take a course, which lasts from 2 to 3 months: 10 drops per day, they will help the patient to get rid of hypovitaminosis, bring the body into tone. You can consume not only vitamins purchased in Pharmacy, but also contained in food. Under this description, vegetables and fruits are fresh: especially in red and yellow. These are grapefruits, oranges, watermelons, grenades, pepper, tomatoes, radishes. Equal gun products - milk, cottage cheese, kefir , sour cream. The basis of the diet is meat - pork, chicken, liver; cheese - melted or cheese; nuts - cashews, hazelnut, peanuts; as well as sea products).
- Lipotropic drugs (they normalize the exchange of lipids and cholesterol in the body, with their help, the selection of fat from the liver is accelerated, as the result of this process, well-fiv-infiltration of the liver decreases. Scales on the skin under the influence of lipotropic drugs are softened. Such drugs are: "Lecitin", " Methionine, "Carnitine", "Inosit").
- Immunotherapeutic preparations (adjust the immune system of the body, which is most often amazed or weakened. These drugs contribute to its recovery, strengthen the protective functions of the body. These drugs include "U - Globulin", "Viferon").
- The use of preparations with iron, calcium, gamma globulin (means in which iron is present) stimulate the immune system, increase the performance of the body, eliminate fatigue. These include "Akiferrin", "Tardiferon", "Ferrum Lek". Preparations containing calcium restore the bone system improve brainwriting, normalize blood pressure, they include: "Calchen", "calcium gluconate". Preparations with gamma globulin perform a protective function in the body, do not affect it with viruses and infections. Such are "Gamma-Globulin Human", "Antigep", "Bianven". All drugs that include the elements described above normalize the functional abilities of the body, which favorably affects skin cover).
- Hormonal drugs (prescribed only in the event of a severe course of the disease, the treatment and dosage diagram is selected by a doctor for each patient individually taking into account the peculiarities of its body. These drugs can be "Tyriodine", "insulin").
- Outdoor ointment and creams (their action is aimed at mitigating skin, restoration of skin functionality. With a given disease, gentle skin care mode is important. Creams need to be applied to clean skin after a shower or bath with a periodicity of 1 or 2 times a day. When choosing Mazes need to know their body for the subject allergic reactions. The most frequently used by ichthyosis are "AEKOL-2", "Mustel", "Unna").
Physiosis treatment with physiotherapy methods
Ichthyosis, the treatment of which can be carried out with the help of specialized baths, has its own characteristics. Therefore, the purpose of all medicines and baths should be based on the diagnosis of the body, taking into account age features and individual approach.
In childhood, the doctor can recommend as a healing skin care of a bath with potassium permanganate. After the bath, the baby's skin is lubricated with children's cream containing vitamin A. For adults, baths with starch and salt content are recommended (salt in the proportion of 300 grams per 200 liters of water is taken, the water temperature does not exceed 38 degrees, the procedure is carried out in no more than 25 minutes with periodicity Less often 2 times a week. The course of therapeutic baths is from 8 to 10). Carbon dioxide and sulfide baths are favorably affected by metabolism in tissues, improve the functionality of endocrine glands. For those who are often resting in the pre-satellites and resort hospitals, peat and ilic mud are perfect.
To date, the treatment of ichthyosis is popularly average ultraviolet radiation. With its help, vitamin D is produced in the human body, the immune system is stimulated, the metabolism and the body is replenished with vitamins A and C.
In addition, it is extensively used in the treatment of ichthyosis of heliotherapy - the impact on the skin with sunlight. As a result, vitamin D is also produced, and the immune system is restored. It is held only at certain daytime hours: morning from 8.00 to 11.00 and in the evening from 16.00 to 18.00.
If you are wondering: is Ichthiosis is fully treated? The answer of dermatologists is obvious - it is possible only to support the significant functions of the body, mitigating symptoms. Adhering to all the advice and recommendations of the doctor, you can maintain the condition of the body at the proper level!
Medicine
The method of prescribed treatment of ichthyosis will depend on several factors:
- Type of disease;
- Severity;
- Patient age.
Depending on the combination of these factors, an outpatient or stationary method of treatment is chosen. At the same time, the treatment diagram is made up of a combination of the following methods:
- Reception vitamin complexes. A sufficient amount of vitamins consumed contribute to mitigating scales, having a lipotropic effect. In mandatory, you need to take vitamins A, C, E, PP and Vitamins of B. The dosage of these vitamins should be from 100 to 120% of the daily rate for this age.
- Reception of immunomodulating drugs is necessary to restore a weakened immune system. The therapy includes drugs containing calcium and iron and gamma globulin. Medicines for ichthyosis include these substances as the main existing components.
- Ichthyosis is often manifested against the background of the disorders of the thyroid gland. To restore the hormonal background, a number of analyzes are performed to obtain information on the state of hormones, then preparations regulate the number of desired hormones towards the norm are prescribed. The change in the hormonal background in a positive side reduces the symptoms of the disease. In hormone therapy appointed by children on breastfeeding, nursing mothers need to be taken similar drugs.
- Taking baths, for the effects of drugs directly on the affected skin. Baths can be taken common and local, depending on which percentage of the body is amazed by ichthyosis. Such preparations such as potassium permanganate, sodium chloride, urea, as well as starch, il, peat and aromatic retinoids are added to the water. Baths are prescribed to almost all patients within the framework of complex therapy. They are very effective, since through sparkled skin cells the drug deep into it and has the therapeutic effect. Which of the listed drugs need to be added to the bath, will determine the doctor for each particular case individually.
- In combination with baths effectively carry out local treatment with creams and ointments. The affected areas of leather need special moisturizes and nutrition, the cream during ichthyosis will help to perform this task. Cream and ointments can also be additionally vitamined and often contain retinol or tocopherol acetate. Ointments have a regenerating effect, invalid and anti-inflammatory ointments are added if necessary. In complex therapy, ointments are used containing D-panthenol as the main active component.
- Also, different types of physiotherapeutic procedures are prescribed to patients suffering from ichthyosis:
- Thalassotherapy is a combination of various drugs of marine origin. It includes swimming in the sea, mudshots using marine healing mud, receiving baths with marine Solu, Algae compresses. The therapeutic effect is due to the large content of iodine, salts and minerals that have favorably affecting the skin.
- Ultraviolet irradiation and helotherapy. With light formats of ichthyosis and appropriate climatic conditions, patients showing a short sunbathing intake, in other cases, special devices for ultraviolet irradiation are used, and the procedure is carried out in stationary conditions. It has an antiseptic and regenerating effect.
Ichthyosis is an incurable disease (with rare exception), but the listed methods will help reduce the manifested symptoms and reduce the risk of complications. The doctor individually selects the complex, which includes several listed techniques, to achieve the maximum result.
Folk remedies
Treatment of ichthyosis at home
Many people with a diagnosis of ichthyosis are asked: "How to treat the disease at home?"
In addition to drug therapy, specialists are recommended to fulfill common procedures: solar baths, water marine treatments (if there is no possibility to visit the sea, it can be replaced by bathing in a bath with sea salt). Baths with the addition of glycerin, borants, soda will be useful (for one procedure). To allow this some result, it is necessary to spend about 20 sessions.
As for various ointments and herbs that are used in the treatment of ichthyosis at home, it is necessary to be extremely careful and with the intention of the use of folk remedies, you must consult with the attending physician. After all popular treatment ichthyosis can not only help, but also harvest the patient. But with the right approach and right combination with traditional medicine The effect will be positive.
To reduce unpleasant painfulness, calm, moisturize and soften the skin, you can use ointment on the indoor medicinal herbalFor this you need to mix:
- 200, the oils of the Zverca
- on 50 g. Pine resin and wax bees;
- for 10 g. Pucked chalk and crushed cleanliness;
- 20 g. Propolis;
- 100 g. Home cream oil.
Take the mass in a metal saucepan on slow fire 2.5 - 3 hours. And after put in a dry and dark place for 12 hours. Then boil again and strain through the gauze. After that, the tool is ready for use, it must be applied to the flabbing areas of the skin 2-3 times a day for 1-2 months depending on the severity of ichthyosis.
Among the medicinal herbs, you can use: Aralia, rowan leaves, pyrzya, sea buckthorn, the plantain is large, Levze, Peony evaded, all these plants when applied will not be damaged. After the preparation of infusion from these herbs, its zest can be used to rub organized skin.
To cure ichthyosis at home, you need to strengthen the patient's immune system, and for this you need to use as much iron as possible, which is in certain food and vitamins.
Summing up the above, the disease ichthyosis cannot be fully cured, but when combined with the right traditional medicine and traditional treatment, It is possible to significantly reduce the area of \u200b\u200blesions and the sharpness of the disease. It should be remembered that any reception of any means must be accompanied by a resolution of the doctor
Information is referenced and is not a guide to action. Do not self-medicate. At the first symptoms of the disease, consult a doctor.
Inheritance of ichthyosis occurs according to an autosomal-recessive type. This is the greatest form of the disease. The newborn has hyperkerate plates in the form of rhombuses, polygons covering the whole body.
Due to the fact that the formation appears in the form of geometric shapes resembling the Harlequin costume, the name of the disease appeared.
Ichthyosis in the photo with a description of 7 pieces
Deals takes a threat to life. Children are born with a small weight. Modern medicine has achieved success in keeping the lives of little sufferers.
If earlier, with a systemic infection, the baby did not survive, as the thermoregulation was disturbed, now medicine is able to preserve their lives.
Views of ichthyosis
- Lamp ichthyosis (lamellar, small scales are formed on the skin).
- Surface (characterized by the presence of erosions, bubbles on the dermis. Other name: Epidermolitic).
- Acquired (formation of scales of different colors, size. They are located on the extensive surfaces of the limbs, cheeks, foreheads).
- Simple (vulgar. Light gray scales appear on the skin, reborn into the brown "shields". Dry skin, peel).
- Ichthyosis of Claiman (found in colloquial speech. But the classification does not mean).
- Cameral ichthyosis (peeling, color change).
- Silent ichthyosis (the secret of the sebaceous glands dries, strongly stands out).
- Silent ichthyosis of newborns (the body is covered with a crust of epithelial cells, hairs, bristle).
- Follicular (appears on the outputs of hair bags).
- Follicular superficial (appears in the form of convex points).
- Snidoid (on the derma there are dense horny shields separated by grooves).
- Ichthyosis on the legs (pthenoid type of disease, similar to deprive. Other types of diseases affecting the body may occur). In this photo, it can be seen how the ichthyosis looks like on the legs:
- Nail ichthyosis (thinned, smell, fragile).
- Bullese ichthyosis (characterized by the appearance of bubbles on the skin).
- Needle (there are formation in the form of spikes).
Causes of ichthyosis
Congenital ichthyosis develops with genetic predisposition.
Acquired ichthyosis has a number of reasons. For example:
- Endocrinopathy.
- Problems in the hematopoietic system.
- Failures in the work of sexual, thyroid glands.
- The adrenal glands only partially function.
- Permanent hypovitaminosis.
- Age changes.
Symptoms of ichthyosis
The result of the disease is to change the structure of the skin. Because of the wrong cell work, due to the loss of memory of the mutating gene (or the presence of another memory), the bark layer is not built as it should be.
The skin, its sections are covered by formations in the form of scales. The disease can be hereditary or acquired. TO characteristic features ichthyosis belongs:
- dry dryness;
- peeling;
- the presence of scales;
- the presence of spikes, plates on a horn layer of gray, brown;
- fucked formations;
- thickening of the skin;
- the film covering the body in newborns (over time disappears);
- shouting nails;
- potted mouth;
- brittle, dry hair;
- multiple caries;
- myopia;
- the thermoregulation is broken.
Treating ichthyosis
What is ichthyosis and how to treat him? Science has not yet found a method for the treatment of ichthyosis of the skin, which can lead to complete healing.
But modern medicine is able to extend the life of a parableous severe degree, improve well-being. A group of doctors works with patients. The team of doctors includes:
- Dermatologist (main courses of treatment).
- Family doctor, therapist, pediatrician (integrated treatment aimed at improving immunity).
- Psychologist (removal of stress, depressive states, getting rid of complexes, clips inherent in patients).
- Otoidolaryngologist (observes the condition of hearing, drummers).
- Dentist (treats teeth).
The group may include those specialists, the assistance of which is required in the fight against the main disease and complications. Patients receive complexes of therapeutic measures aimed at facilitating the state of the skin with ichthyosis.
Mediosis treatment preparations
This includes ointment, creams, gels that are applied to the skin. They help to cleanse sections from scales, facilitate the course of the disease.
The skin must be moistened and soften, as the cracks are formed, in which the infection may fall. Therefore, the drugs of antimicrobial, antifungal action are selected.
Also, they choose moisturizing creams, gels for a soul with therapeutic effect, ointment with urea.
Hydrotherapy (hydrotherapy)
What else can you treat the skin ichthyosis? A good effect is given bath. Usually apply:
- salt;
- starch;
- with potassium parchment solution;
- sea;
- carbon dioxide.
It is a good result of appliqués using peat, illy mud. As a result of treatment, the metabolism in the body is improved, in the skin itself.
Vitamin therapy
Patients are offered vitamin and mineral complexes. They are produced in the form of capsules. Therapy is aimed at improving immunity. Preparations act as common means.
Prolonged use of vitamin A, its analogues (retinoids) B, C, E, PP leads to mitigating scales, skin cleansing, increase its elasticity. The drug appoints a doctor in a small dosage, since side effects are possible.
Diet and therapy (proper nutrition)
In the diet of the patient, it is necessary to introduce foods rich in vitamin A, B, E, C. They will get rid of hypovitaminosis, which refers to one of the causes of ichthyosis. The menu must attend:
- Dairy products.
- Meat.
- Yellow, red fruits, vegetables.
- Seafood.
- Porridge.
- Dried fruits.
Vegetables, fruit should not overstat the menu. This can cause allergies.
Heliotherapy
Treatment with the Sun, its infrared radiation is shown for illness suffering. To do this, there are special devices that collect solar rays into the bundle. He is sent to sick places.
Thalassotherapy
Naval climate treatment involves not only sea bathing, air walk. The use of algae, seafood enrich the patient's menu, will saturate the body with a set of beneficial substances.
Enveloping therapeutic mud is also shown in such a diagnosis.
Climatotherapy refers to alternative treatment methods. This method is proposed to the world by naturopaths. Treatment not only removes symptoms, but also strengthens the psyche, raises the mood, improves the state.
The cell is built up DNA chains. When mutations occurring in the gene, the skin remains without a healthy horny layer, as the gene does not remember how it is built.
But he remembers how scales are built, that is, the skin of the inhabitants of the underwater world. So the skin is arranged for the underwater, amphibian inhabitant.
Treatment of ichthyosis by folk remedies
At home in the treatment of ichthyosis, herbs are used. Therapy is aimed at removing symptoms, improvement of well-being:
- Remove itching, pain help infusions. Make a row, wipe. Suitable as raw materials: dyeing, nettle, oats, rowan (berries), plantain, ivy.
- The ointment of pine resin, bee wax, clean, chalk, hunter, butter, creamy oil, propolis.
- Prepare fees from the Pijmas, mother-in-law, horsetail, plantain, crawling pottery. Apply inside, drink courses for 30 days.
- Herbs from fees can be combined with vegetable, olive oil, smear amazed places.
How else can you treat ichthyosis of the skin at home? You can make herbal, salt baths, rinsing with drier.
Prevention of ichthyosis
If the parents of the future baby have mutating genes, they should appeal to genetics. He calculates, a future newborn will appear with pathology or not. The carrier can be one parent.
If a woman is already in position, she can do ultrasound. Some doctors are sent to the biopsy, where the scraping from the skin of the fetus is taken. It may be unsafe for the future kid. The disease of the child is diagnosed when he is still in the womb.
Is the ichthyosis infecto?
Most often, ichthyosis is transmitted by inheritance. The acquired form appears with reduced immunity, age-related changes.
It is impossible to infected them, since it is a violation of metabolic processes in the body. The skin increases the number of amino acids, as the protein and fat exchange is incorrect.
Is it possible to cure ichthyosis of the skin?
Unfortunately, scientists have not yet fully understood in pathological processes that occur at a given ailment. He is incurable. There are a number of methods that facilitate the course of the disease.
Is it possible to cure ichthyosis light shape? This is possible if the disease is purchased, not launched, treatment began immediately.
Only on very small areas of the horn layer may remain milded, dry, brown formations.
The treatment of ichthyosis has many methods in arsenal.
How to get rid of ichthyosis?
As soon as the process of genetic mutation becomes clear, scientists will immediately be able to offer a decision on the treatment of ailment to complete healing. Until then, it remains to wait for research results. Modern medicine reached a lot, but not everything can not yet.
The first sign of the development of ichthyosis becomes dry skin. The skin becomes crash. Over time, it is compacted and cracks, and white or gray crusts appear on its surface. On the surface of the palms and feet, the skin pattern becomes more pronounced. Nails in the amazed areas are thin and weak, easily break. The hair is almost not growing, and if they are still growing, then very weak and rare.
More photos of ichthyosis are not for the faint of heart. To view - click on plus \u003e\u003e\u003e 
With ichthyosis, the child can lose weight, there are violations of mental development, endocrine diseases. The level of immunity in such children is much lower than that of peers. Also observed chronic disorders view: conjunctivitis, myopia.
Such violations are most pronounced under the age of 10 years. In the later age category, the severity of the disease is much lower, due to the change of the hormonal background. A distinctive feature of this disease is the period of its exacerbations. Pathology is usually sharpened in winter, with cold dry air. In summer, especially with high humidity, the condition of patients is dramatically improved.
Note: In order to treat, many patients change their place of residence. In the conditions of a warm humid climate, the disease practically does not make itself felt.
Symptoms and signs of ichthyosis
Symptoms of ichthyosis depending on its variety are somewhat different. The scales on the skin can be of various shapes and size, located differently in the parts of the body and arise in different periods of life of the patient.
Symptoms of ichthyosis - Table
| A variety of ichthyosis | The period of the first symptoms of the disease | Exterior skin | Location of horny scales on the body surface | Blowing hair, nails and other organs |
| Ordinary ichthyosis |
| Keroderma:
|
|
|
Brilliant ichthyosis:
|
||||
| White ichthyosis: white scales | ||||
| Sail-shaped ichthyosis: large gray-colored scales | ||||
| Simple ichthyosis: scales small, tightly attached to the base | ||||
| X-linked ichthyosis (synonymous disease: black ichthyosis) | The first weeks of life | Scales Large, Dark or Almost Black | Throughout the body with the exception of palms and soles |
|
| Intrauterine | Horny shell from large scraps (shields) gray-black | Throughout the body |
|
|
| The first days of life |
|
|
|
|
| Congenital ichthiodine erythrodermia (Brock's disease) | The first days of life |
| Throughout the body |
|
| The first days of life |
| Throughout the body, especially in the skin folds (groin, elbow and popliteate venge) |
|
|
| One-sided ichthyosis |
| Red leather shade |
|
|
| The first days of life |
| Throughout the body |
|
Varieties of ichthyosis - Photo Gallery
With vulgar ichthyosis, the whole surface of the skin is affected
In epidermolytic ichthyoma, the skin impresses asked
The ichthyosis erythrodermia of Brock is a kind of congenital ichthyosis
With lamellar ichthyosis, the skin is covered with dense flakes in the form of shields
For ighthyosis, horny arrangements are characteristic of
In some cases, ichthyosis is part of the changes in the body caused by the broom of the genes. The result is the sustainable combinations of skin phenomena and some other abnormalities of the structure and the work of the internal organs.
Hereditary diseases accompanied by the development of ichthyosis - table
| Name pathology | Type of inheritance | Symptoms |
| Netherton syndrome | Autosomal recessive |
|
| SHEGREEN-LARSSON SYNDROM | Autosomal recessive |
|
| Refsum syndrome | Autosomal recessive |
|
| Ore syndrome | Autosomal recessive |
|
| Popov syndrome | Unknown |
|
Clinical picture of individual forms of ichthyosis
Simple, ordinary, or vulgar ichthyosis
It is the most common form of the disease. It does not appear immediately at birth, but in the third month of life and later - up to 1 year. There is no sexual difference in the frequency of morbidity.
Clinical symptoms of this form of the disease:
- flywood, hebuguene and fine-plane peeling;
- follicular hyperkeratosis;
- hair loss;
- severity of palm and plantar skin patterns; Sometimes, especially with the accompanying pathology of the endocrine system, there is peeling on the soles and palms;
- dryness, roughness, deformation, thinning and fragility of nail plates.
The lesion of the skin in the form of pronounced dryness and roughness is generalized, with the exception of the side surfaces of the face, cervical, axillary and berry regions, the inner surface of the hips, bending surfaces in the area of \u200b\u200bthe knee and elbow joints.
The painting of scales is different - from whitish to grayish black. The most pronounced changes are noted in the zone of knees and elbows, and on the front surface of the shins, the scales are similar to the scales of fish.
The defeat of the settlement of the settlement hair follicles In the form of follicular keratosis is the most characteristic symptom of ordinary ichthyosis. It lies in the blockage of the mouth of small traffic jams consisting of the masses of the horny epithelium, as a result of which the nodules are formed, the tubercles with a size of 1 to 3 mm with twisted powder hair in the central part. The tubercles have a bodily or reddish-gray color, covered with scales and are sometimes surrounded by a reddish wedge.
Follicular keratosis gives the skin a grungy look (symptom of "graters") and can be localized throughout the skin of the skin, except palms and stop, but most often in the field of shoulder belt, buttocks and hips. It is especially expressed in adolescence and leaves for middle age after himself point atrophic slaves, which are the only testimony of ichthyosis in family members.
Rezidiosis recurrences occur during dry air and during the cold season. With age, there is an improvement in the state, especially in the summer or / and in a wet warm climate. Often in adolescence comes a period of short-term remission. In the future (by 25 years), the peeling becomes much less pronounced or stopped completely, but the characteristic changes of the skin of the palms and stop are preserved.
Follicular ichthyosis
Follicular ichthyosis differs from follicular hyperkeratosis as a symptom of vulgar ichthyosis, or Darius disease (follicular vegetative keratosis), in which 4 forms are distinguished. Unlike vulgar ichthyosis, Darya's disease develops in childhood or in adolescence, has a progressive nature, exacerbates under the influence of ultraviolet radiation. The nodules have the nature of dense warts growing papules, with a merger of which in the folds of the skin arises a mocking. Elements are usually located symmetrically. Their main localization is the moral part of the head, the existed area, face, inter-opaque zone, region of the sternum and large folds.
X-hooked ichthyosis
They suffer mainly boys. The carrier of the pathological gene is mother. Girls can get sick if the father sick, and the mother is a carrier of the gene. The disease can be diagnosed already at birth (rarely) or after 2-6 weeks. Less heavy forms are manifest later, but up to 1 year after birth.
Clinical symptoms are inherent only for this form of the disease. Large scales are tightly adjacent to the skin surface and have a dark brown (dirty) color. Characteristic lesion location - rear necks, side surfaces chest, leather extensitive surface limbs.
A significantly less specific character of hyperkeratosis has in the field of natural skin folds. The face, palm and soles are not affected, but the phenomena of hyperhydroposis are noted in these departments. Flushing of flour type and follicular keratosis for this form are not characteristic.
In 50% of patients, corneal turbidity is revealed, which is not accompanied by a decrease in visual acuity. In addition, sometimes leather pathology is combined with other disorders - with cryptorchism, with hypogonadotropic hypogonadism, with retardation of mental development. In female carriers of a mutated gene, the development of primary generic weakness is possible.
The course of the disease worsens in the winter season. The clinical picture as the age increases does not improve.
It occurs with the same frequency among female and male. More often found in children born from parents who are close relatives. Often these children die still at the age of the newborn.
The disease is manifested immediately at birth with brilliant thin dense film of the horn layer of the epidermis having a yellowish brown color. Film, due to which similar newborns got the name "colloidal fruit", covers the whole body. During the movements of the child, it cracks and for several weeks is separated in the form of thin plates of large sizes. At the same time, salted red skin sections are exposed.
With age, the intensity of the red color decreases, but the degree of large-scale peeling increases. The plates themselves have a polygonal shape and a grayish brown. They are tightly fixed in the central part, their edges peel and raise, like tile.
The defeat is diffuse. In the elbow and knee areas, the skin is thickened, sometimes bearded expansions of the epidermis, and on the back, due to the resulting folds, the skin acquires a transverse waviness. Palm and plantar surfaces are also susceptible to redundant orog.
In addition, itching and hair loss on the type of edge alopecia, nail plates, on the contrary, thicken and grow rapidly. Due to the tightening of the skin, "Mongoloid" eyes are formed, ear-sinks are deformed.
Mortality in this form reaches 20% mainly due to the blockage of sweat glands, excessive moisture loss in the field of multiple cracks, increasing body temperature to high rates Even with minimal physical stress or increase the ambient temperature, the addition of secondary infection and the development of septic conditions.
Diagnostic events
The correct diagnosis is made after a thorough study of the skin of the infant and carry out appropriate analyzes, as it is very often a congenital form is confused with the dermatitis of the ritter and the erythroderma leiner-mousse.
Deskvamative erythrodermy leiner-mousse can be found in the first two months of the life of the baby: the epidermis is inflamed and slightly eaten, the skin folds are peeling. And literally in a few days, all skin covers are involved in this process.
Ritter dermatitis develops on the seventh day after birth. Red epidermis, flakes in the area of \u200b\u200bthe natural holes of the baby (navel, anal hole). The process further applies to the whole body, and scales begin to disappear with the formation of erosions. Additionally, the body temperature increases, dyspeptic phenomena and toxicosis are attached.
X-clutch and ordinary shape can be confused with gluten disease, which is also characterized by the peeling of the epidermis, the violation of the structure of the nail plate and the hair follicle.
Related problems
Little children and adolescents poorly tolerate the disease. The reasons are to fail the work of the body systems:
- The child grows slowly, does not gain weight, looks awkward, often the disease is accompanied by flaws in mental development.
- The disease enhances the selection of sebum, it disrupts the structure of the hair, they can even fall out. The baby's nail plates become thin and fragile.
- Immunity is weakening, and therefore viral diseases often develop.
- The disease provokes disruption of metabolism, the work of the endocrine system and hormonal exchange.
- Cholesterol is significantly increased, which leads to a disease of the heart and blood vessels.
- The "chicken blindness" is developing - a disease in which a person does not distinguishes objects in the dark.
Problems are especially pronounced in winter. Treatment must be accompanied by proper nutrition and hygienic skin procedures. You can not exclude daily walks. Treatment also requires sunbathing.
Forecast:
The forecast depends on the form of ichthyosis. With ordinary, lamellar and X-clutched recessive ichthyosis, it is usually favorable for life. Dispensary observation in a dermatologist, prophylactic treatment in the autumn-winter periods by a complex of vitamins in combination with UV Oblueny, sanatorium-resort treatment contributes to maintaining the skin of patients in a relatively favorable state. With innate I., especially combined with defects of nervous and endocrine systems, lethal outcomes are often noted, due to violation of the development of vital organs and systems.
Diagnosis of the disease
Ichthyosis has so pronounced symptoms that a specialist can easily determine the disease. It is similar in its manifestations with seborrhea and psoriasis, therefore differential diagnosis A specialist dermatologist is engaged.
After inspection of the skin, the doctor will appoint several additional surveys:
- general and biochemical blood tests;
- general urine analysis;
- scraping epidermis.
If the parent suffers from ichthyosis or in the family there are patients with this disease, doctors offer to make a special analysis of the fetal skin. The child in the womb takes a biopsy of the skin. You can spend the study at about 20 weeks of pregnancy. Unfortunately, other methods of diagnosis in relation to a child who has not yet been powerless. If the result is positive, the specialist will propose to interrupt pregnancy.
Ichthyosis is not transmitted to children from patients with parents only in 3% of cases. That is why doctors recommend to patients with these diagnoses to refrain from the birth of children.
Treatment
Genetic forms of ichthyosis are not amenable to complete cure, and with the forms of this dermatological disease, the therapy of the main illness, which caused this pathology is shown the patient. Currently, studies are underway to create drugs that would be able to influence the changed genes and contribute to their recovery. It is possible that their creation will allow patients with hereditary forms of ichthyosis to get rid of this unpleasant and severe disease.
Treating all forms of ichthyosis is carried out by a dermatologist. The need to hospitalize the patient is determined by the severity of the clinical case.
The following drugs can be assigned to patients with various forms of hereditary ichthyosis:
- vitamins A, C, E and group B;
- a nicotinic acid;
- hormonal drugs: insulin, thyroid hormones, glucocorticosteroids (according to indications);
- mineral additives with potassium, wick, iron;
- preparations with aloe;
- immunomodulatory funds;
- plasma transfusions and the introduction of gamma globulin;
- antibiotics (when the secondary infection is attached);
- oil solution of retinol (in the form of droplets when the age of twist).
For local treatment, patients with ichthyosis can be assigned:
- the use of special means for overall hygiene and skin care: gel, cream and losterine shampoo;
- lipotropic preparations with lipamide and vitamin U;
- baths with manganese, decoction chamomile, yarrow or sage;
- applying a children's cream with vitamin A;
- therapeutic salt, carbon dioxide and starch baths;
- applying creams with auevit, urea, vinylin and sodium chloride (for adults);
- mud;
- ultraviolet irradiation (in suberymmetric doses);
- impact by seaweed, water or other products of the sea.
- reception infusion of phytosbolation from nettle, oats grains, rowing flowers, rowan fruits and grass, mother-in-law and plantain;
- reception infusion of phytoschera from the grass of the mother-in-law, Pijmas, plantain, the horsetail and root roots;
- ointment based on the oil of the Zverboy, wax, propolis, gils, cleanliness and butter;
- baths with influences of medicinal herbs (needles, tea, calendula, hay Truchi, etc.);
- baths with soda, brown and glycerin, salt and brown.
The treatment plan of ichthyosis is compiled for each patient individually and depends on the shape and severity of the disease. In addition to complex overall and local therapy, such patients and their families show work with a psychologist, since this disease is always associated with a violation of psychological equilibrium. In turn, the timely elimination of stresses, internal experiences and problems with alienation from society has a positive impact not only on the patient's psyche state, but also contributes to improving general status patient. Permanent psychological support prevents the faster progression of the ailment, which can provoke an unfavorable emotional background.
The forecast of ichthyosis in most cases is unfavorable, since, even with the easiest course of this disease, systemic pathologies often join, and progression exchange violations leads to the development of complications. The only way to prevent the hereditary forms of ichthyosis is to plan pregnancy with the obligatory determination of the risk of birth of a child with such pathology.
Params in which this pathology is observed from one of the parents, it is recommended to refuse to born their own children, ECO services using donor material (egg or sperm) or adoption.
When identifying the anomalies of the fetus, her artificial interruption can be recommended during pregnancy.
Forecast and Complications of Ichthiosis Harlequin
The prognosis is unfavorable: 50% of newborns die in the first few days after birth. Those who survived the critical moment will later fight:
- eye complications (as a result of a resistant chance of a century);
- slowing down growth;
- violations of motor abilities;
- psychological problems and the lack of social skills.
Long-term reception of retinoids can give complications on the liver, kidney, a cardiovascular system. Unfortunately, the lives of people with such a diagnosis as a whole are associated with huge torments, but medicine does not stand still, and every day new techniques appear, significantly improving the patient's condition.
The main causes of ichthyosis of the skin
At the heart of the epidemiology of ichthyosis of the skin is a gene mutation that is not fully studied by science and medicine. Doctors and scientists are not able to say with accuracy, which causes the development of ichthyosis, but with accuracy confident that the disease can develop against the background of the following factors:
- heredity;
- violation of exchange processes;
- the pathology of the thyroid gland;
- disorders of the functionality of the genital glands, adrenal glands;
- shift in the work or formation of cellular immunity.
In cases where the skin's ichthyosis acquired, other pathological disorders in the body may become its cause: cancer, chronic renal failure, thyroid disease. Not rare, ichthyosis of the skin flows together with the accompanying dermatological diseases of the chronic flow: eczema or other forms of dermatitis.
Symptoms and varieties of vulgar ichthyosis
There are two varieties of Ichthyosis vulgaris:
- autosomal dominant;
- recessive, associated with floor (x-chromosomal).
These varieties differ in the clinical picture.
Autosomal dominant form Means that mutated genes are inherited independently of the floor (both men and women can be carriers of mutation). Initially, the skin of the newborn is not damaged - the symptoms are manifested at the end of the first year of life, sometimes - a little later (in the second year).
Characteristic signs:
- the presence of yellow or brown spots (formed from the horny cells of the surface layers of the epidermis) in size from several millimeters to several centimeters, which resemble fishing scales;
- changes, as a rule, are located within the chest, back, upper and lower limbs (symmetrically);
- in general, coverings on the whole body become dry due to reducing the secretion of sebum and sweat, follicular keratosis is often observed on the legs and buttocks;
- on the skin of the head, there are also characteristic details, but the hair is not damaged.
Often the disease coexists with follicular keratosis, hyperkeratosis of internal surfaces of hands and legs and atopic dermatitis.
It's important to know! Ithyosis is vulgar in autosomal-dominant-type children spontaneously disappears with the onset of puberty. That is, even if you do not spend pharmacological treatment, The baby will turn out this disease.
Recessive (x-chromosomal form) It is found exclusively in men (women are only carriers of a defective gene) and manifests itself at the moment of birth or the first months of life. Scale changes affect the whole body, as well as the area in armpits, groin, fiba of the elbow joints and the popliteal area. Symptoms deteriorate with age.
In addition, patients are observed:
- violation of vision (inflammation of the cornea, cataract);
- problems with the musculoskeletal system (incorrect development of cartilage and bone tissue, muscle atrophy);
- hearing loss;
- violations by the central nervous system (muscle spasms and mental retardation).
It should be noted that the recessive form meets very rarely - 1 case by 6000 people.
Causes of the diagnosis of ichthyosis
The main causes of the appearance of this disease - in the human body, the generation of genes occurs or a violation of their expression - transformation of hereditary information in proteins or ribonucleic acid. All these changes in the functioning of the body are inherited. Depending on the form of the disease, these changes in humans flow in various ways. Defective keratin can be observed, the lack of such a product as sterling sulphate, as well as the hyperplasia of the basal layer of the epidermis and other similar processes.
Acquired ichthyosis is a rather rare case. It may arise due to lack of vitamins, problems with thyroid gland or adrenal glands.
Causes of follicular surface ichthyosis
Follicular keratosis is multifactorial diseases. From the main reasons you can allocate:
- Avitaminosis of fat-soluble vitamins (mainly a and e).
- Nutrition of food: an unbalanced diet, the absence of regular meals, excess in it carbohydrates. Insufficient water consumption.
- Excessive stress, psycho-emotional overvoltage.
- Reception some medicines (glucocorticosteroids, genital hormones, pituitary hormones).
- Sigor weather conditions.
Follicular surface ichthyosis arises against the background of too rapid epidermis energization in separate skin sections. Keratin, which is part of the horny layer of the skin, clogs the mouth of the hair follicles, forming the so-called "tube", which rises above the body surface.
Ichthyosis in the photo with a description of 7 pieces
Deals takes a threat to life. Children are born with a small weight. Modern medicine has achieved success in keeping the lives of little sufferers.
If earlier, with a systemic infection, the baby did not survive, as the thermoregulation was disturbed, now medicine is able to preserve their lives.
Classification of acquired ichthyosis
- symptomatic, provoked by any pathology or is one of the signs of the underlying disease; They may be disorders of the function of the thyroid gland or adrenal glands, the dysfunction of the neuro-vegetative system, hypo or avitaminosis "a", blood disease and malignant neoplasms, autoimmune, inflammatory and infectious diseases, metabolic disorders, intolerance to individual drugs or food, etc. d.;
- discoid;
- singile, or senile.
In accordance with the classification proposed in 1990 (K. N. Suvorov), which is based on clinical symptoms, distinguish such forms of the disease as:
- simple, or ordinary ichthyosis, characterized by the defeat of all skin cover and small scales;
- brilliant - also affects almost all skin covers, excluding large folds, but transparent with a gray-shaped flakes are mosaic;
- the serpentine, in which the skin of the extensive surfaces of the limbs and side surfaces of the body are affected, large scales have a grayish brown color.
As a basis for another classification, the severity of the disease is taken, according to which the form of the latter is determined:
- severe, the outcome of which is the death of a newborn baby;
- medium-heavy, compatible with life;
- late when the first symptoms of pathology appear only on the second month after birth.
These and other classifications in various cases can be convenient in practical work, but in the international classification of ichthyosis diseases are placed under the heading "Other congenital anomalies" and are classified as "congenital anomalies, deformations and chromosomal violations". In the same classification, they are divided into ichthyosis:
- Simple (vulgar) is an autosomal dominant type of inheritance.
- Captured with X-chromosome (female) - recessive.
- Plate, or lamellar - autosomal-recessive.
- Congenital bullous ichthiodine erythrodermia - autosomal dominant.
- Fruit ichthyosis is an autosomal recessive.
- Another congenital ichthyosis.
- Congenital ichthyosis is unspecified.
The mutated genes transmitted by inheritance control enzyme systems and biochemical processes of keratinization, which are not fully decrypted. They are based on the pathological development of the profilrangin - the cellular protein contained in keratinocytes. Normally, as a result of biochemical processes, it disintegrates on philhargin and molecules complex, which is called a moisturizing factor (NMF).
As a result of the gene mutation, a disorder of skin moisture occurs, excess synthesis of defective keratin, which is the protein of hair, leather and nails, an overly fast epithelium epithelium. The violation of exchange processes, especially protein and fat, leads to a violation of the barrier function of the skin and accumulating in the blood of cholesterol, and between the cells of the horizontal and other layers - various sets of exchange products, in particular, the amino acid, which have cementing effect. Also slow down the horn layer is slowed down, as it is tightly tight with the subject-based layers, the processes of sweating and respiration of the skin are disturbed, local immunity is reduced. Given the large area of \u200b\u200blesions, all this may not be reflected on the functions of neuro-endocrine and other organism systems.
Thus, the main reason for most forms of ichthyosis is the mutation of genes or violation of their development. It is assumed to mutate at several points of one gene or participating in this process of several different genes, which causes the presence of a large number of clinical forms of the disease.
Pathological oroging is developing at the following mechanism:
- Excess keratin products, as a rule, with a modified structure.
- Accelerated movement of keratinocytes to the horn layer from the basal.
- Strengthening bonds between the horn layer cells and their delayed rejection.
- Dystrophic changes in the epithelium, formation of vacuoles (bubbles) in the upper layers of the epidermis and an increase in the thickness of the horn layer.
- Philhargin and NMF formation violation, leading to excessive water loss through skin. This causes their dryness and characteristic peeling.
Treatment with folk remedies
Possible treatment of follicular surface ichthyosis by folk remedies. Most popular such recipes:
- Cut the thick sheet of aloe, wrap with thin paper and freeze for up to 3 days. Cut into thin plates and apply to the affected places at night under a gauze bandage.
- Lubricate the skin-affected propolis.
- Grate one young potato on a shallow grater, apply the resulting cascum-shaped mixture to the affected areas for three hours three days in a row.
Folk treatments
How to treat ichthyosis using folk remedies, will correctly explain the phytotherapist. In the treatment of pathology, the following plants were well shown: sea buckthorn, Pijm, Horseta, Mother and Levsay, Sowing Oats and Rowan, Peony, Eleutherokok Spiny, Dressee and Aralia High. You can take or one species, or several types of medicinal plants: Pijma - two parts; Cardiac dye - one piece; Plantain - two parts; Horsetail - one piece; Drub - four parts. The mixture is poured with hot water (500 ml), is insisted. Use infusion at half the cup 2 times a day. Reception - 1 month. Further 10 days - a break. The course should be repeated.
The first symptoms and the treatment of windmills in children and adolescents
Causes and factors of development
Most of the forms of ichthyosis are hereditary diseases. All information about the person is enclosed in the DNA molecule. Its individual sites - genes are responsible for the specific aspects of the body's activities. Ichthyosis - the result of a breakdown of the DNA section, which manages the update of the epidermis layer. The transfer of the wrong gene by inheritance is different with different forms of the disease. One incorrect gene leads to the appearance of the following types of ichthyosis:
Such an inheritance mechanism is called an autosomal dominant. As a rule, the disease can be traced in the pedigree in each generation. With an autosomal-recessive type of inheritance, the parents of the child are healthy, but each of them handed its wrong ichthyosis gene. The probability of birth of a sick child in this case is less than in the previous one. Parents often do not suspect the carrier of incorrect genes, since ichthyosis can only manifest itself in some generations of pedigree. By this mechanism, the following types of ichthyosis are inherited:
In some cases, the irregular gene of ichthyosis is located in the X chromosome. In such women, the manifestations of the disease are not observed because they have a normal gene in the second X-chromosome. In men, X-chromosoma is one inherited from the mother. If there is an irregular gene in it, the boy develops ichthyosis. Girls in such a family do not hurt, but transmit a defective gene to offspring (carriage).
Mystery DNA - Video
Apparently, several genes are controlled by the oroging of the top layer of the skin. When they breakdown, change on the skin and inside the body are similar, however, they differ in some features. Normally, the rate of oroging the top layer of the epidermis is comparable at the speed of updating other - granular, brilliant and peeled. With ichthyosis, this situation changes dramatically. The lower layers grow with the usual or slow motion. The horny layer is growing extremely quickly, so many times superior in thickness normal. In some cases, ichthyosis can develop for reasons not related to heredity:
With ichthyosis, the nature of the skin is changed. Normal scales are so small that indistinguishable with a unarmed look. With ichthyosis, the epidermis is healing with the formation of large very dense scales of various shades, connected to the bottom layers of the base. Between the rows of scales are formed deep cracks. The whole picture resembles a fish with cushion or crocodile skin.
Ichthyosis is not only a cosmetic problem. The whole organism suffers from changes from the skin. Cracks between scales - wide gate for infection. Normally, the skin protects a person from entering the fabric of pathogens of microbes. This contributes to the integrity of the cover and friendly bacteria that dwell on the surface under the closer control of immunity. With ichthyosis, individual flakes are not an obstacle to microbes, so the disease is often complicated by inflammation of deep tissues and the penetration of the pathogen into blood (sepsis). Scaly skin is not able to perform another important work - maintain due temperature inside the body. For patients with ichthyosis is dangerous and overcooling, and overheating.
Plate ichthyosis - video
Common forms of ichthyosis
There are several varieties of ichthyosis, the manifestation of which depends on hereditary factors. Here is some of them:
- ichthyosis harlequin;
- lamellar;
- vulgar;
- epidermolitic;
- x-hooked.
Sometimes this diagnosis is also called the fetus ichthyosis. Children with this diagnosis, as a rule, appear on light premature and with a slight weight. Externally, the diagnosis is manifested not only by the peeling of the skin, but also by changing and redness of the eyelids, the ears, mouth, the restriction of the movements of the joints of the arms and legs. The skin of a newborn is covered with gray or brown scales with thickening and cracks. 
Such a disease appears due to mutation of the ABCA11 gene - its polypeptide chain is shortened, as a result of which irreversible changes occur during the formation of the fetus. As a result of a similar process, the functionality of lipids is broken - they are not capable of forming a horn layer of the skin. Ichthyosis is most often visible after birth, but, as a rule, during the ultrasound, some signs of developing deviations can be traced, especially if parents have a hereditary predisposition. A ultrasonic examination of the fetus state is required - the development of the mouth, ears, nose, the face profile is estimated, the edema of the limbs is also possible.
Most often, the outcome of the disease is unfavorable - newborns with such a diagnosis rarely survive. In some cases, timely therapy can extend the child's life for a while.
Most often, a similar diagnosis is made immediately after the birth of a child. The skin of the baby looks bright red - erythrodermy, there is a film on it, which makes it difficult to breathe and eating a child. This condition is also called colloidal fruit. After some time, the film turns into a scaly, which remains for life or disappears in childhood, without leaving complications. If the flakes did not disappear, then in adulthood they increase in size, while the red skin falls. In the footsteps or palms there may be painful cracks, on the face - a slight peeling. It is possible to develop dementia - acquired dementia. 
Often, the presence of a film on the body of a newborn is accompanied by a change in the eyelids and lips, which can also be maintained throughout life. The reason for such a diagnosis is also the hereditary mutation of genes, which can manifest itself in persons both male and female.
Such a disease is manifested in the period of life from 3 to 12 months, both in boys and girls. It is accompanied by horny tubes on hair follicles and a decrease in the grainy layer of the skin - the value of keratogial ripen in its cells is reduced. For diagnosis, histological examination and typical history will be required. 
Vulgar ichthyosis is manifested by dryness and peeling of the skin in the area of \u200b\u200bthe forearm, back, butces. In this case, irritation is absent on the buttocks, the inner surface of the hips, under his knees and armpits. The most pronounced stage of the disease - in the period of puberty, with age, the manifestation of the disease decreases. Also, the aggravation of the disease occurs during the cold winter period, in the warm mild climate, the symptoms of the disease become less pronounced.
This diagnosis is often called congenital bullous erythrodermia of Brock. It manifests itself immediately at birth or in the first months after childbirth in the form of bubbles of different magnitude with specific contents on red skin, which over time is open, forming erosion, and with time epithelisites. Scales usually linear shapes and dark shades may be located in large folds or neck. Between the affected areas may freeze healthy skin, which will be one of the symptoms of epidermolytic ichthyosis. With age, the number of bubbles may decrease, but it will increase the amount of peel or scales. 
In case of infection, the patient's condition may deteriorate on the skin. Typical for ichthyosis changes in the eyelids - their withdrawal is not observed. An additional symptom can serve blessed legs of the feet and palms with the effect of thickening. With the defeat of only these parts of the body, it is possible to manifest another type of ichthyosis with various mutation of genes. To clarify the diagnosis requires an external examination, history and results of histological research.
This type of ichthyosis is manifested in male people in the first months of life. Most often, it looks like large skin scales in the form of black or dark brown plates. Sometimes a similar scaled can be linked to each other, creating a layer of damage in the form of a shell. Education is observed on the back surface of the neck, the scalp, buttocks and hips, and are absent on the face, feet and palms. In some cases, the revival of the cornea is possible, so in addition to a dermatologist's doctor, a comprehensive inspection of profile doctors is sometimes required, including an ophthalmologist. 
To confirm the diagnosis, blood test may be required, as well as family genetic information and general history. The relief of the disease can occur in the summer period of the year, when in the warmth, wet climate. Frosty dry air can provoke aggravation of the disease. As a rule, with age positive dynamics is not observed.
Ichthyosis recognition methods
The diagnosis of ichthyosis is a task for an experienced dermatologist. The following methods are used to establish the right diagnosis:
Differential diagnosis of ichthyosis is carried out with skin diseases accompanied by redness, appearance of bubbles and other defects:
How to treat ichthyosis skin
At the moment, ichthyosis cannot be cured, you can only reduce the intensity of symptoms, and this significantly affects the quality of life. It is also necessary to treat the accompanying pathologies.
Attention! Treatment should be under the strict observation of a dermatologist. It can occur both in stationary and outpatient conditions. It all depends on the severity of the disease.
The main methods of treatment of ichthyosis:
- correction of a hormonal background;
- preparations local action;
- physiotherapy;
- vitaminotherapy.
If bacterial infection is attached to ichthyosis, the course of antibacterial drugs is appointed. They must be seamless with the antibioticogram.
We will also consider the treatment of ichthyosis by folk remedies that are able to facilitate the patient's condition.
Hormonal background
Be sure to do the correction of the hormonal background. The thyroid, pancreas affect ichthyosis.
In hypothyroidism, thyroid drugs are prescribed - thyroidin, thyroid, thyreokomb. In case of lack of pancreas use insulin. The treatment diagram is selected strictly individually.
Local action preparations
Means for humidifying skin, exfoliation of horny scraps are used.
- Ointment Vaseline. Moisturizes and softens the skin. It has a dermatoprotective effect. Applied to the affected cleaning skin.
- Dermatol cream. It has a membrane protector, prevents inflammatory processes, moisturizes, increases the elasticity of the skin. Satures its lipids, has an antiallergic action. It is applied to the affected area of \u200b\u200bthe skin with a thin layer 2 times a day. The course is two to three weeks.
- Salicylic acid (solution). It has anti-inflammatory and keratolytic effect, disinfects. Alcoholic leather is processed 2 times a day.
- Ointment acrider. It has anti-inflammatory, antiallergic, anti-ethnic effect. Applied thin layer 4-5 times a day. When defeating the face, the course of treatment should be no more than 5 days.
- Diprosalik ointment. It has anti-inflammatory, anti-face and vasoconstrictor action. It has a disinfecting and keratolytic action. It is applied with a thin layer in the morning and in the evening.
- Ointment Vidniem. It has a membrane protector effect, reduces the process of skin oroging, saturates with vitamin A tissue, contributes to the process of regeneration of the skin. Applied thin layer 2 times a day. Before using Mazi, affected places are processed by an antiseptic. Depending on the severity of ichthyosis, the course of therapy is 1-3 months.
Physiotherapy
Physiotherapy procedures moisturize the skin, reinforce the metabolism in the tissues. A positive effect can be noticed a week after the start of treatment.
Attention! Procedures must choose a specialist based on the form of ichthyosis and the degree of its severity.
The following physiotheraces are useful:
- Healing baths. Contraindicated in acute inflammatory process or exacerbation of chronic disease, with blood diseases, oncology. There are oxygen, hydrochny, starch baths.
- Measual-wave ultraviolet irradiation (Suf-irradiation). Contributes to the formation of vitamin D, as well as its isomer - vitamin d2; Normalizes the metabolism, saturates the organism with vitamins C and A. increases the concentration of certain biologically active substances that directly affect the health of the endocrine system. Stimulates immunity.
- Thalassotherapy. The use of various seas or ocean factors (algae, sea water, il, healing dirt). Combination various methods Thalassotherapy leads to long-term skin moistening and a decrease in the severity of symptoms.
- Heliotherapy. Solar radiation is used for therapeutic purposes (solar baths). The procedure contributes to the formation of vitamin D, stimulates the immune system. It is conducted in a strictly defined time: in the morning - from 8 to 11 hours, after lunch - from 16 to 18 hours.
Vitaminotherapy
Patients are prescribed water-soluble and fat-soluble vitamins in large doses. This reduces the severity of some symptoms.
- Vitamin A (in the form of ointments).
- Vitamin E (solution for intramuscular injections).
- Vitamin B1 (solution for intramuscular injections).
- Vitamin B6 (solution for intramuscular injections).
- Vitamin B12 (solution for intramuscular injections).
What it is? Drakes call skin lesions arising in the area of \u200b\u200bthe skin folds. Specialists ne.
The development of pathology Child disease scabies today is often found quite often. The highest risk of infection
Hytiosis Harlequin (the fruit of Harlequin, Lat. Ichthyosis Fetalis) is a rare genetic disease belonging to the group of general modelmatosis, and inherited by autosomon-recessive type. This is the most severe form of congenital ichthyosis, which is characterized by the presence of a newborn hyperkerate plates of a rhombid and polygonal form. They cover the whole body with a geometric pattern resembling Harlequin's costume (hence the name of the disease). In addition, there are other changes that carry a direct risk of life. Children with Hytiosis Harlequin have low birth weight. Previously, they quickly died due to the attachment of a systemic infection or as a result of thermoregulation disorders. Modern medicine has achieved success in the fight against the symptoms of this ailment, so there is a chance to preserve the life of the newborn.
It is difficult to calculate the exact frequency of occurrence, doctors believe that it is 1: 1000,000 cases. In closed communities (for example, in tribes) ichthyosis of the type of harlequin is diagnosed more often, because there is a higher probability of marriage unions, in which both partners are carriers of a mutant gene. Pathology is equally striking female and male babies.
Causes of Hytiosis of Harlequin
So, we dealt with the concept of Hortyosis of Harlequin, what is the disease. Now consider exact reasons.
The gene mutation shocks the polypeptide chain in the ABCA11 gene, which causes the loss of the protein function that transports lipid granules into keratinocyte membranes. Microscopically was found that with this pathology, grainy membranes are incorrectly formed, so lipids (fats) cannot create the correct horny layer and are exposed to abnormal secretion. Even in the intrauterine age (about the 16th week), the fetus develops Hortyosis of Harlequin. We cannot eliminate the causes of mutation, but then the future mother can learn in advance about such a problem and consider abortion.
Symptoms and clinical painting of Hortiosis Harlequin
Symptoms are present from the moment of birth. The kid has observed:
- thickened leather with large brilliant diamond or polygonal flakes; These flakes have light color and separated by bright red cracks;
- heavy ecredopion (reversing) of the upper and lower eyelid;
- redness of the eyelids;
- flange lips (lips turn), mouth open: a child for this reason can not take parental milk;
- pseudo-Morphia of the face (secondary symptom with respect to skin lesions): flattening of the nose, small rudimentary lines, in some cases ears and nose can be completely absent;
- contracture of joints, difficulties with flexion and extension of the limbs caused by damage to the dermis;
- little head (microcephaly);
- in some cases, the underdevelopment of the fingers and legs, the nail hypoplasia, polydactilation (more than five fingers on hand);
- structural anomalies of the central nervous system, which can manifest itself with convulsions.
Diagnosis of Harlequin's ichthyosis
Most often the diagnosis is carried out after birth, on the basis of clinical picture. If parents have a genetic predisposition to this mutation (there were similar cases in the family), it is advisable during an ultrasound to look for signs that may indicate the fetal ichthyosis:
- flat face profile;
- flat nose;
- wide "fish" mouth, macrogloss (large language);
- flat ears or their absence;
- swelling hips and legs;
- slow fetal growth.
Listed symptoms are not specific, and may indicate other health problems. Therefore, if the doctor doubts, he can prescribe a biopsy of the skin.
listed symptoms are not specific, and may indicate other health problems. Therefore, if the doctor doubts, he can prescribe a biopsy of the skin.
Treatment of ichthyosis harlequin
The child requires an interdisciplinary approach that includes consultations of many specialists - an oculist, a surgeon, a gastroenterologist, a nutritionist, a psychologist. In order to provide proper nutrition, he can install gastrostomy.
Therapy includes:
- moisturizing the child's body;
- supply of opioid analgesics, for example, morphine, fentanyl, sufferentanyl (in order to reduce painful reaction to tissue damage);
- introduction of Etretinat - Retinoida for and other scaly-like diseases;
- the use of softening agents for skin and mucous membranes;
Appointment of antibiotics to prevent infection;
Psychological assistance.
Thick crawled plates will gradually peel and disappear. After a few months, a thin dry skin of bright pink (places covered with small scales will appear in their place. This means that the baby survived the critical stage of his life, and can be completed intensive therapy. However, patients with a diagnosis of ichthyosis harlequin treatment will last a lifetime. It consists in constant body care - the use of keratolics to remove scales and softening creams to reduce dryness.
Treatment with folk remedies
Folk medicine did not find effective funds from this pathology. But herbal baths are used to care for undercurrent leather (with oat straw, core loving, black grass, chamomile flowers) and compresses. Based on swine fat and medicinal herbs, nutritious and wound-healing ointments are made. Remember that before using phytotherapy it is better to consult with a dermatologist.
Forecast and Complications of Ichthiosis Harlequin
The prognosis is unfavorable: 50% of newborns die in the first few days after birth. Those who survived the critical moment will later fight:
- eye complications (as a result of a resistant chance of a century);
- slowing down growth;
- violations of motor abilities;
- psychological problems and lack of social skills.
Long-term reception of retinoids can give complications on the liver, kidney, a cardiovascular system. Unfortunately, the lives of people with such a diagnosis as a whole are associated with huge torments, but medicine does not stand still, and every day new techniques appear, significantly improving the patient's condition.
Prevention of Hytiosis Harlequin
There are no ways to prevent this ailment and future child. However, before entering into marriage, you can check yourself and the future husband / wife on genetic mutations (there is a special test). If both spouses have deviations on the ABCA11 gene, one should consider the issue of marriage, or to refuse to conceive children.
Photo
Ichtiosis disease Harlequin photo
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Ichthyosis is a hereditary skin disease, which is characterized by a violation of skin destruction processes.
As a result of this disease, the skin of the patient is covered with scales, externally resembled Czech fish. For Ithyosis is characteristic of its early beginning (the first 3 years of life). Ichthyosis leads to a rich peeling of the skin, which looks like fishing scales (from Greek. Ichthys - "Fish"). The severity of the symptoms of the disease depends on the set of factors. Sometimes ichthyosis practically does not give discomfort and a person can live with him all his life. But in sometimes congenital ichthyosis can lead to severe damage to the child's skin, which can cause fatal outcome (fetal ichthyosis).
Different forms of ichthyosis arise from a person due to genetic mutations, which have not yet been fully understood. Recently, the main cause of this disease is called vitamin A insufficiency, as well as the emergence of diseases of the endocrine system. The main pathological process is excessive generation of keratin (skin protein).
Types of ichthyosis:
The following forms of ichthyosis distinguish:
- vulgar ichthyosis;
- lamellar ichthyosis;
- X-hunty ichthyosis;
- darius disease;
- ichthyosis erythrodermia.
Vulgar ichthyosis
Vulgar, or it is also called ordinary, is the most frequent form of ichthyosis. According to statistics, vulgar ichthyosis occurs in people about 75 - 95% of cases among all forms of ichthyosis. In most cases, children in the first years of life (from 1 to 3 years) are sick of this form.
The main reason for the occurrence of vulgar ichthyosis is a genetic mutation that is inherited by autosomal dominant type. This type of inheritance suggests that the genetic disease will manifest itself in a child when he inherits a mutant allele (one of the forms of the gene, which leads to an alternative development option) at least from one of its parents. It is important that this type of hereditary disease does not depend on the floor of the child and manifests in girls and boys with the same frequency.
Pathological disorders are generalized and applied to almost all skin. The skin becomes very dry, peeling appears, and the skin thickens. Skin glands (sweat and greasy) practically do not function. The main feature of ordinary ichthyosis is the defeat of the extensive surfaces of the upper and lower extremities. The most often affected by the extensive surface of the elbow, ankle and knee joints, as well as the sacrum area. Children often amazed face skin - forehead and cheeks. Depending on the severity of the disease, horny flakes can be transparent and thin or dark and massive. With vulgar ichthyosis, plugs of horny scales are often formed, which can clog the holes of hair follicles (follicular keratosis) and sebaceous glands. The mechanical pressure of these horny tubes leads to atrophy (reducing the number of functional cells) of the sebaceous glands and hair follicles. With such a clinical form of ichthyosis, hyperkeratosis is observed (thickening of the horn layer), as well as a decrease in the grain layer of the epidermis. In addition, degenerative processes occur in the sprout layer of the epidermis.
It is worth noting that with such a form of ichthyosis, the flexible zones of the elbow joints, the axillary depressions, the popliteate pits, as well as the groin zones are not affected.
The main clinical forms of vulgar ichthyosis include:
- Kerodermia.KSERODERMIA - is the most easily flowing form of vulgar ichthyosis. Kerodermia leads to dryness and insignificant skin roughness, which most often appears on the extensive surfaces of the limbs.
- Simple ichthyosis. Simple ichthyosis is characterized by unbearable symptoms. The main symptom is dry skin, combined with its peeling. Horn scales look like bran (seven-party scales). The difference from Keroderma is that not only limbs are affected, but also torso. Often you can see the defeat of the scalp. Horny flakes have small sizes, and in the center they are tightly connected to the deep layers of the epidermis.
- Brilliant ichthyosis. Brilliant ichthyosis is characterized by a large accumulation of translucent scales on the extensive surfaces of the limbs, which are especially much in the hair follicle zone. Rarely horny scales look like a mosaic.
- Snidoid ichthyosis. Sail-shaped ichthyosis is characterized by the appearance of ribbon-like brown or gray scales. With snake-shaped ichthyosis, horny scales are surrounded by deep furrows that resemble snake cover.
Note that the symptoms of ichthyosis becomes weaker during puberty due to the change in the hormonal background. This genetic disease lasts all life. The exacerbations of ichthyosis occurs in winter.
Lamellar ichthyosis
The lamellar ichthyosis appears due to the defect of protein of transglutaminase. This protein regulates the process of differentiation of keratinocytes. The transglutamine protein defect leads to hyperplasia (excessive growth) of the basal layer in which is observed fast growth Keratinocytes and their transition from the basal layer to the corneum.
With lamellar ichthyosis, the child's skin is red and completely covered with fine yellow-brown film. Sometimes this film is transformed into large horny flakes, which completely disappear after a short period of time. But most often the scales remain in a person for the rest of his life.
As a rule, under lamellar ichthyosis, hyperkeratosis with age is only enhanced. At the same time, the regression of erythrodermia is observed (redness of the skin decreases). The strongest lesions occur in the skin folds. The skin on the flag in most cases is red, stretched and peeling. As a rule, heightened sweating, especially on thick areas of the skin.
X-hooked ichthyosis
X-hosted ichthyosis is a recessive skin disease. This type of disease is striking only men, and women can only be carriers of a defective gene. X-linked ichthyosis leads to congenital insufficiency of steroid sulfate. This enzyme is needed to transform steroid hormones from an inactive form to active.
According to statistics, X-hosted ichthyosis occurs approximately one of 2,500 - 6,000 men. This form of ichthyosis has been manifested for 2 weeks of life. X-hosted ichthyosis is externally similar to the serpentine ichthyosis - there are many dense horny scales of dark brown color, around which cracks and furrows are formed. Often, boys with x-clutch ichthyosis are diagnosed with mental retardation, as well as different anomalies for the development of skeleton bones.
Dorier's disease
Daria's disease (follicular discratosis) is one of the clinical forms of congenital ichthyosis. Daria's disease leads to a delay in the mental development of the child, and also affects the thyroid and sex glare.
Daria's disease is characterized by a gyperplazia of a grain and horn layer. As a result of a defect of one of the enzymes involved in the process of keratinization, abnormal tales and grains appear in the spike layer.
The main symptom of the follicular discretosis is skin rash with a scaly surface. The skin forms many dense nodules (papules) of spherical shape. Papul size, most often does not exceed the floor centimeter. Very often, the nodules merge, forming mocking foci. It is also characteristic of the development of sub-hyperkeratosis, which leads to the thickening of the nails. It should also be noted that with Dari disease, mucous membranes may be affected.
Ichthyosis erythrodermia
The ichthyosiforous erythrodermia (Brock's disease) is characterized by the synthesis of defective keratin, as well as a violation of the growth and differentiation of keratinocytes. In the upper layers of the epidermis, a large number of grains and vacuoles are formed (bubbles), and a strong thickening of the horn layer (Aktokateratolitic hyperkeratosis) occurs. One of the differences between the ichthiosiforous erythrodermia from vulgar ichthyosis is the presence of inflammatory infiltrate in the derma (leukocyte accumulation).
The ichthyosis erythrodermia is an autosomal dominant disease. It leads to thickening and skin edema. A lot of bubbles and mocking foci appear on the skin. Also for broken disease, inflammatory phenomena are characterized, which occur in the dermis. Nails, more often, thickened and deformed. It is worth noting that the symptoms of the disease with age are most often regressing (decrease).
Symptoms of ichthyosis
Symptoms of ichthyosis are very much and they are diverse. E each clinical form ichthyosis There are a number of symptoms of characteristic manifestations. In this case, the severity of symptoms depends on the severity of the disease:
- Skin dryness is a direct consequence of violation. water salt balance. In case of disease, the epidermis is not capable of holding fluid. Dry skin directly depends on the amount of lost fluid.
- Peeling - appears due to excess detachment of horny scales from the surface of the epidermis. With ichthyosis, the speed of the desquamation of cornecitis is much increased.
- Redness of the skin (erythrodermia) - occurs during ichthyosis erythrodermia. Skin color can vary greatly and depends on the severity of erythrodermia.
- The deformation of the nails is due to a defect in the synthesis of keratin (the main protein of the nail plate). Nails can thicken, peel, and sometimes significantly deform. It is not necessary to note that the defeat of the nails is not necessarily for vulgar ichthyosis.
- Thinning and hair liability - occurs due to atrophy of the hair follicles and the effects of horny stoppers. Thinning hair also appears due to a defect in the synthesis of keratin, which is in the hair rod in large quantities.
- Piedermia (purulent-inflammatory skin disease) - it occurs secondly due to the decline in local immunity. For pathogens of microorganisms, the affected skin is very vulnerable. Most often, the pyoderma arises against the background of microeraumamism of the skin, as well as in the event of the appearance of itching.
- Conjunctivitis - various ichthyosis forms can appear different eye lesions. The most frequent form, this is the inflammatory process of the mucous membrane of the eye (conjunctivitis). The inflammatory process at the edges of the eyelid (blepharitis) is not rarely observed and in the retinus (retinit).
- The pronounced skin pattern on the palms and footsteps is one of the most frequent symptoms of ichthyosis. The pronounced exhaustion of the skin pattern indicates a hereditary skin disease.
Important! In the fetus ichthyosis, children are often born much more than a period of time, such newborns are not always viable.
Causes of ichthyosis
Ichthyosis is a hereditary disease, so the main reason for this skin disease is a gene mutation transmitted from generation to generation. Biochemistry mutations are not deciphered now, but it is known that the disease is manifested by a violation of fat and protein metabolism. The result of this pathology is that excess amino acids and cholesterol accumulates in the blood, this leads to the appearance of a specific skin reaction. In patients having a gene mutation, which leads to the development of ichthyosis, is observed:
- violation of the thermoregulation of the body;
- slowing down metabolic processes;
- increasing the activity of enzymes that take part in oxidative skin respiration processes;
In patients with ichthyosis, there is also a decrease in the work of the endocrine glands - genital, thyroid, adrenal glands. These symptoms may appear immediately or increase gradually as the disease develops. The result is that the patients grow the deficit of cellular immunity, the ability to absorb vitamin A is also reduced and the activity of sweat glands is violated. This increases the chance to detect the diseases of the sweat glands as a hydrocystoma, siringoma, the equally spiradenoma.
Important! If the patient is diagnosed with the acquired form of ichthyosis, it should be sent to an additional examination. The purpose of these surveys should be the elimination of malignant neoplasms.
Treating ichthyosis
The treatment of ichthyosis is appointed by a dermatologist. The treatment of therapy depends on the form of the disease and the severity of the flow. Most often, treatment is carried out outpatient, but with severe forms of the patient's disease, can be placed in the hospital.
The following techniques are suitable in the treatment of ichthyosis:
Vitaminotherapy. Patients with ichthyosis disease are prescribed courses of vitamins of groups A, B, C, E, as well as nicotinic acid. Vitamins are prescribed in large doses (it depends on the age of the patient). Reception of vitamins has the softening of scales on the skin.
To increase the immunity in patients with ichthyosis, the reception of iron and calcium preparations, aloe extract, gamma globulin is prescribed.
Patients with ichthyosis are prescribed blood plasma transfusions.
With poor work of the thyroid gland, the pancreas, adrenal glands characteristic of this disease, the doctor picks up hormone-plating therapy. For the treatment of severe ichthyosis, especially in newborns, hormones are used.
Children who are twisted in the eyelids are prescribed an upset of oil solution of retinol acetate. The dosage of hormones and the reception schedule is prescribed by a doctor for each patient separately, it depends on the severity of the disease. Nursing mothers of patients with ichthyosis of children are appointed the same treatment as their babies.
Under the local treatment of ichthyosis in children, it is understood as the purpose of baths with a solution of potassium permanganate. Adult patients are prescribed baths (local or common, depending on the localization of skin lesion) with the addition of sodium chloride, starch, urea.
To soften the skin during ichthyosis, the doctor prescribes the use of ointments and creams, which includes lindamid and vitamin U.
Patients with ichthyosis also shown physiotherapy. In this disease, effective procedures are the procedures for thalassotherapy, ultraviolet irradiation, carbon dioxide and sulfide baths, heliotherapy. During the periods of remission of the disease for preventive Mer Put peat and slim baths, and it is also necessary to use aromatic retinoids.
Treatment of methods of traditional medicine
Along with the therapy, a prescribed doctor, for the treatment of ichthyosis, it is recommended to additionally apply drugs prepared on the basis of medicinal herbs.
Lowing methods of traditional medicine
To improve the status of the skin during ichthyosis, folk healers are recommended for patients:
Little sun bath. Take them in the morning to 11 hours.
Local action preparations
For the treatment of ichthyosis, various local action preparations are used, which are able to moisturize the skin, and also contribute to the exfoliation of horny scales (keratolytic action).
- Vaseline is an outdoor ointment. It softens and moisturizes the skin in the affected places, while it has a moderate dermatoprotective effect.
- Dermatop - Cream for outdoor use, which has a membrane protector (protects the membranes and epidermis cell organelles). It also reduces the production of biologically active substances that run the inflammatory process. Dermatop affects the epidermis, moisturizing it, and also increases the elasticity of the skin. To the measurement, the drug saturates skin lipids and has a moderate antiallergic effect.
- Salicylic acid is a solution for outdoor use, which has a moderate anti-inflammatory effect and has a keratolytic and disinfecting effect.
- Acriderm - ointment ointment, which has a pronounced anti-inflammatory effect by reducing the synthesis of inflammation mediators (cytokines). It also has a moderate anti-essay and anti-allergic effect.
- Diprosalik - ointment ointment. Betamethazone dipropionate has anti-inflammatory, vasoconstrictor and anti-face effect.
- Modify - ointment ointment, it has a membrancrotective effect, contributes to the process of regeneration of the skin and significantly reduces the oroging process. When it penetrates the skin deep, it contributes to saturation of tissues with vitamin A.
Physiotherapeutic treatment methods.
Sometimes physiotherapy procedures use for the treatment of ichthyosis. Such procedures help moisturize damaged skin, and also enhance the metabolism in tissues. The effect of treatment is observed after 1 week of course. It should be noted that physiotherapeutic treatment methods must necessarily be selected and appointed by a doctor who will proceed from the clinical form and the severity of the disease.
The following physiotherapy procedures are used to treat ichthyosis:
- Therapeutic baths calm and have a good effect on the nervous and cardiovascular system of man. Therapeutic baths improve the metabolism of the affected areas of the skin. Depending on the main component exist different types Medical baths. It is important that the appointment of therapeutic baths is contraindicated in an acute inflammatory process or exacerbation of chronic disease, with blood diseases, as well as oncological diseases.
- Oxygen baths are a variety of gas baths. The main substance is oxygen located in dissolved form. The water temperature in the bath must be from 34 to 36ºС. A 200 ml of 2.5% of the potassium permanganate solution (manganese) is added to the water, after that it is necessary to thoroughly stir water. Then add 40 ml of 20% sulfuric acid solution and 200 ml of hydrogen peroxide solution, after mix. After 10-15 minutes, the patient can take an oxygen bath. The duration of the procedure is from 15 to 20 minutes. Course treatment: 12-15 baths.
- Salt baths are the most simple in preparation. For the preparation of the salt bath, it is necessary to take 250 - 300 g of salts for 200 liters of water (bath with a very low salt concentration). Water temperature: no more than 38ºС. Duration of the procedure: 20-25 minutes. Course treatment: 10 baths.
- Starch baths consist of fresh water, the main component of which starch is served. To prepare a bath, add 1 kg of starch to the water, whereas for the local bathing bath 200 g. Pre-starch mixed with a small amount of cold water, and then pour this mixture into the bath. Water temperature: from 34 to 36ºС. The duration of the procedure: not more than 25 minutes for adults, and for children - no more than 10-12 minutes. Course treatment: 10 baths.
- Measual-wave ultraviolet irradiation (SUF-irradiation) in suberies and erythemical doses have a beneficial effect in the treatment of ichthyosis. This physiotherapeutic procedure helps to form vitamin D in the skin, as well as vitamin D2. In addition, the SUF-exposure stabilizes the metabolism and saturates the body with vitamins A and C. Currently, it is proved that the SUF-irradiation increases the concentration in the body of certain biologically active substances that directly affect the effect of the endocrine system. Also, medium-wave ultraviolet irradiation helps to increase the resistant functions of the body and stimulates the human immune system.
- Thalassotherapy is a complex method of improving the entire body, which includes the use of various factors of the sea or ocean (therapeutic dirt, sea water, algae, Il). IN sea water It contains multi-forming elements and macroelements, such as iodine, iron, calcium, manganese, phosphorus, fluorine, they are needed for the proper operation of various organs. The combination of several methods of thalassotherapy leads to long-term humidification of the skin, which contributes to a decrease in the severity of symptoms.
- Heliotherapy is based on the use of solar radiation in the therapeutic purposes. Heliotherapy helps the formation of vitamin D and stimulates the immune system. This procedure is done using sunbathing. Patients are located on high topsters, the head must be covered with a headdress or keep in the shade. In order for the effect of solar radiation to be symmetrical and uniform, it is necessary to periodically change the position of the body. Heliotherapy is carried out in a strictly defined time. In the morning from 8 to 11 hours, in the afternoon from 16 to 18 hours. Air temperature: not lower than 20ºС. The first session of heliotherapy: no more than 15 minutes. Every 3 days it is necessary to increase the stay under the sun for 3-4 minutes. After 1 week, it is necessary to take a break in 2 days. The maximum time of irradiation with sunbeams: not more than 1 hour.
Important! If the family has cases of ichthyosis, the determination of the degree of genetic risk is mandatory.
Preparations
Photo ichthyosis
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The consequences of ichthyosis
The forecast for ichthyosis is almost always unfavorable. This is due to the fact that even with a light form of ichthyosis, systemic pathologies and progression of violation of exchange processes begins, which leads to the development of severe complications.
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Prevention of ichthyosis
Since ichthyosis is a hereditary disease, the prevention is mainly consulting a genetics doctor even before pregnancy.
What is ichthyosis?
Ichthyomy is a skin disease, due to a violation of the energization processes of skin cells transmitted by inheritance. The disease is the brightest representative of a group of skin dermatosis diseases.
It looks like peculiar flakes on the skin, resembling fish Czech. Amino acid accumulations with cementing properties begin to form between these scales. So, the scales turn out to be tightly connected with each other, because of which the separation of them from the body becomes rather painful.
This disease is similar in symptoms on kerata diffuse, hyperkeratosis, various kinds of dermatosis. In addition, about 20 species of this disease are distinguished in the world, with similar symptoms, but various forms of manifestation.
Read more about photos, symptoms and treatment of skin kerats in adults in another article.
You can cite the following classification of forms of ichthyosis:
- simple, with the defeat of all skin of small scales (vulgar ichthyosis);
- transparent or brilliant, with damage to the skin with transparent gray scales, in appearance resembling mosaic (lamellar ichthyosis);
- screering - on the skin thick horny plates, superimposed on each other (Hythyosis of harlequin, epidermolitic ichthyosis of the late form);
- x-hosted or black, with consistent intense damage to the skin with dense scales of dark color.
From the stage (complexity) of the development of this disease, many factors depend on:
- external and internal signs of illness;
- the complexity of the diagnosis of the state of the patient and the form of the manifestation of ichthyosis;
- the appointment of a certain treatment.
There are several degrees of complexity of skin ichthyosis:
- Easy shape. This is the late manifestation of the disease, starting aged 3 months to 12 years.
- Medium-heavy shape. The disease proceeds from birth, but children survive.
- Heavy or historicoid form. Newborn dying for several days of life.
Causes of skin ichthyosis
The main reason for the appearance of this skin disease is a violation in the body of a protein metabolism. This occurs due to the accumulation of a large number of amino acids, which, in turn, leads to a violation fat Exchange, enhance cholesterol and, subsequently, to a gene mutation that causes ichthyosis.
The reasons affecting such gene changes of the body are mainly internal:
- hormonal disorders and diseases of the endocrine system;
- avitaminosis, in particular vitamins of group A;
- increased in the blood of the "harmful" cholesterol;
- skin age changes;
- asocial lifestyle.
Forms and symptoms of ichthyosis of the skin
Vulgar ichthyosis
Another name is ichthyosis ordinary. The most common form, turning over the autosomal dominant type. It is detected in children to three months of life, but can progress until three years. It affects the skin on any part of the body, except for the groove zone, axillary and patellied depressions, elbow folds.
Symptoms:
- It begins with dry and rough skin, which is gradually covered with small white or gray-haired scales. It also deteriorates the condition of the hair, they become dry and brittle, caries appears, nails are smeared, conjunctivitis develops.
- Progresses the disease depending on the degree of gene mutation than it is more, the heavier stage of ichthyosis. With a light form represented by only dryness and a small peeling of the skin, an abortive course is possible.
- Ichthyosis, as well as any disease, leads to a decrease in the body's immunity, as a result, open spaces for the development of allergies or purulent infections. Progressing, the disease also damages the cardiovascular system and affects the liver.
Congenital ichthyosis
Congenital ichthyosis (Hortiezina), respectively, its name is developing in the mother's womb, about 4-5 months of pregnancy. The child is already born with skin covered with thick (up to 1 cm) with horny-black chain shields, also in the intervals of the swamped by furrows and cracks.
Because of the dense clutch of scales, the external infant external organs are exposed:
- the mouth is either strongly stretched or, on the contrary, it is narrowed so that it barely passes the probe for feeding;
- ear holes have an unnatural shape;
- eye eyelids - twisted.
The consequences of congenital ichthyosis are related pathologies:
- skeleton disorders - closure and kopeur;
- interfallated jumpers (membranes) on palms and feet;
- no nail.
The disease can cause premature genera, increases the risk of the birth of a dead fetus. Due to the presence of anomalies incompatible with life, children after their birth usually die in the first days of life.
Lamellar ichthyosis
Also has the name "plate". It manifests itself from birth, as it also treats congenital, and it takes very hard.
A child is born completely covered with large scaly platinum, which make up a kind of "shell". Most of the symptoms are similar to the symptoms of congenital ichthyosis. There is a slowdown in the processes of flow and salo-waste. Because of the cover of scales, hair is rare.
Lamellar ichthyosis is often accompanied by various disorders in development, such as:
- deafness;
- blindness;
- dwarf and others.
Recessive ichthyosis
Recessive ichthyosis (X-hosted ichthyosis) is solely in men, however, inherited by the X-chromosome. Its cause becomes a defect of placental enzymes. Diagnosed from the 2nd week of life, in the most exceptional cases - earlier.
The skin is covered with large dark brown flakes in the form of peculiar flaps. In the intervals, the skin is made by cracks, which externally makes it similar to the snake. In children with this disease, mental retardation is usually observed, the improper structure of the skeleton, epilepsy. In some cases (10 - 12%) hypogonadism or juvenile cataracts appear.
Epidermolithic ichthyosis
Epidermolithic ichthyosis is one of the forms of congenital ichthyosis. All skin of a born baby has a bright red color, as if she was thrown by boiling water. With the slightest touch to it, scales depart.
In some cases, hemorrhage is observed in the skin and mucous membranes. With this coincidence, children are most often dying. In easier cases of epidermal ichthyosis of the skin, the affected surface becomes less, but during life the disease occasionally sharpens, usually with an increase in body temperature to critical numbers.
At about a three-year-old age in places of natural skin folds, layers are beginning to form in the form of thick dark gray scales.
Together with epidermal ichthyosis, the following pathologies may be diagnosed:
- diseases of the endocrine system;
- cardiovascular diseases;
- pathology of the nervous system;
- oligophrenia;
- spastic paralysis;
- anemia;
- infantilism and others.
All of them further complicate the flow of ichthyosis, because of which the percentage of mortality increases. In the world, the most common forms of skin ichthyosis - vulgar and recessive.
Photo
Diagnostics
Dermatovenerologist is engaged in diagnostics. Due to pronounced symptoms, this disease is not difficult to diagnose this disease. The differential diagnosis with similar dermatological diseases is becoming significant: psoriasis or seborrhea.
To determine the degree of progression of ichthyosis and its form, clinical studies are also appointed:
- general blood and urine tests;
- blood chemistry;
- histologic analysis (skin scraping).
In cases where the child is encouraged by the parents, one of which is a carrier of the disease, it is necessary to make a biopsy of the fetal skin, which is the only way to diagnose ichthyosis. This study is carried out at the 19th-21st week of pregnancy.
Treatment
Skin fluid is not subject to complete cure. One can only facilitate the course of the disease and reduce the frequency of exacerbations. With any form of ichthyosis, only complex comprehensive treatment will help, together with constant proper skin departure.
A complex of dermatovenerologist can be registered with next drugs and procedures:
- vitamins of groups A, E, PP, C, B, as well as drugs based on them;
- corticosteroid hormones;
- lipotropic drugs, to reduce skin orog;
- immunotherapy;
- donor blood plasma.
Hormonal drugs are prescribed with severe manifestations of ichthyosis, and, consequently, with its innate forms. With a positive result of such treatment, gradually reduce the reception of these drugs.
Also hormonal drugs are used in the treatment of heavy forms of bubbles.
To the appointment and reception of hormonal should be treated with special attention, given, in addition to the form of the disease, the patient's age, the mass of his body, as well as other features of its body.
With ichthyosis, it is impossible to engage in self-medication. Many drugs have toxic properties and complex side effects. Therefore, this issue needs to be completely rented on a competent specialist and constantly be under its supervision.
Physiosis treatment also includes special external skin care, which needs constant moisture:
The above procedures can also be conducted only after consulting a doctor.
In the treatment, various kinds of physiotherapy can be appointed, aimed at stimulating metabolic processes in epidermis tissues:
- ultraviolet irradiation;
- mud;
- carbon dioxide;
- heliotherapy.
The appointment of inpatient treatment is necessary in case of severe disease.
It includes:
- hormonal therapy;
- physiotherapy;
- immunotherapy;
- treatment with medicines, receiving vitamin complexes.
Treatment at home contains:
- medication therapy;
- external skin care.
Description of the disease
Ichthyosis disease is the name of a rare genetic skin disease. Since ichthyosis is a genetic mutation, this disease is not contagious - you cannot "catch" it during communication with the patient.
The disease ichthyosis causes dead skin cells to accumulate in large-scale areas on the surface of human skin.
In most cases, ichthyosis, these areas are soft to the touch and they are only in specific areas of the body. However, in some cases, skin lesions in ichthyosis are serious and can cover large areas of the body, including abdomen, back, arms and legs.
Ichthyosis (Sauriasis) refers to a group of genetic disorders inherring on autosomal dominant type. It is characterized by suddenly manifesting the upper layers of the epidermis. Localization of oroging may be the most diverse and highlight the exact system in this case is hard enough.
The reasons
Vulgar ichthyosis refers to the simplicity of ichthyosis, the development is directly related to the presence of gene changes in the body and it is inherited by an autosomal dominant type of inheritance.
At the same time, mutated genes are inherited, enzyme systems and the process of skin keratinization can control. Fully this mechanism is not deciphered.
The physiological keratinization of epithelial cells and the formation of the skin horny layer proceeds "softly", consist in dividing the embryonic cells and their movement to the upper layers of the skin.
The lower layer of fresh epithelial cells under normal conditions gradually displaces the old one, which is accompanied by a usually inconspicuous peeling.
Thus, this life cycle, which lasts for two days, forces the newly formed epidermis cells to move to the skin surface and contributes to the transfer of all substances that are contained in them.
The consequence of the violation of the keratinization processes, leading to various skin diseases, called keratosis, or hyperkeratosas, is a slow rejection of the epithelium or / and a pronounced increase in the thickness of the horn layer.
A huge variety of features of clinical manifestations, a variety of morphological picture, the presence of rare forms, the lack of an unambiguous view of researchers for the causes and mechanisms for the development of hyperkeratosis, to which a large group of diseases associated with the term "ichthyosis" also include clinicians to develop a certain unified classification.
The reason for the development of congenital ichthyosis is always mutations of genes that are inherited. Factors provoking such gene violations have not yet been studied by geneticists.
It is known that mutations lead to a change in many biochemical processes in the patient's body, which are manifested by a disruption of the deafness of the skin.
As a rule, signs of congenital ichthyosis are observed in a child immediately after the appearance of it into light, but sometimes are noticed for the first time in childhood.
To date, the causes of lamellar ichthyosis are not fully identified. However, the most real reasons are violations in the process of keratinization of the epidermis.
This can occur due to gene mutations that can be inherited. At the same time, if direct descendants of parents who suffered from lamellar ichthyosis, this disease was not identified, their children are no longer potential carriers of a mutated gene.
Lamellar ichthyosis can most often develop with a pronounced transglutamine protein defect, which regulates the process of formation of keratinocytes.
It also leads to an excessive breaking of the basal layer with an active growth of keratinocytes, which leads to their promotion to the horn layer from the basal.
Also K. probable reasons The appearance of this skin pathology includes the following factors:
Pathology is a hereditary disease (in the overwhelming majority). Exact etiology is currently unknown.
In the patient's body is disturbed by the synthesis of amino acids. As a result, they begin in excessive amounts to accumulate in the blood, which leads to a violation of keratin synthesis.
Also, such a picture is accompanied by a disorder of the thyroid gland, hypofunction of genital glasses.
Over time, a steady violation of homeostasis is developing. This is accompanied by a violation of the absorption of vitamin A, which is necessary to maintain the health of the skin.
As a result, it fails in the work of sweat glands. The oroging of the upper layers of the skin is developing, which over time is exacerbated to the state of hyperkeratosis.
Other types of ichthyosis
In addition to ordinary (vulgar) ichthyosis, there are also other types of this disease, which differ from each other with symptoms, severity, the condition of the skin coating.
In general, they are about forty. The most common is aburient, black, white, needle, simple and snake-shaped.
The treatment of ichthyosis of the skin is prescribed depending on the type and form.
The most common form of ichthyosis of the skin, ichthyosis is vulgar, is inherited by dominant type. It is manifested in early childhood, in the form of dryness and rough skin, most often on the elbows and knees.
The skin of the face is usually not amazed in childhood, and in adults appears peeling on the skin of the forehead and cheek. There is a peeling of palms and soles.
Nails brittle, hair can thin.
There are several types of vulgar ichthyosis: Kerodermia (light dry dryness and roughness of the skin), ichthyosis is simple (skin lesions and scalp), ichthyosis brilliant (thin transparent scales on the limbs), ichthyosis white (white scales, leather, as well as flour), ichthyosis Snidoid (large gray-brown scales).
X-recessive ichthyosis is found only in boys, often accompanied by the development of the development of the CNS, the skeleton.
The separation by classification of this skin disease is carried out depending on the severity:
- a severe form in which the symptoms are very obvious, noticeable with the external examination of the patient's body. It is also called a historicoid form. It can be characterized by the emergence of a premature child to the light, while such children die during the first days of life;
- medium-heavy shape - children, as a rule, survive;
- late form of the disease - disease manifestations are detected with some delay in time.
Clinical signs
The disease of ichthyosis, the treatment of which requires immediateness, begins to appear from the first months of life. It can be evidenced by rough and dry skin covered with tightly surrounding gray-black or whiten flakes.
The groin zone is not affected by ichthyosis, the area of \u200b\u200bthe axillary depression, patellied pate, elbow folds.
Palm palm is covered with mucoid scales, a pronounced skin appears. The severity of the course of the disease depends on the depth of the gene mutation, the possibility of abortive flow.
Nails, teeth and hair in ichthyosis are subjected to dystrophic changes. Skin disease is accompanied by chronic retinitis and conjunctivitis, myopia, allergic diseases of chronic nature, disorders of the internal organs (liver problems, cardiovascular failure).
During the diagnosis of "ichthyosis", treatment should be comprehensive and aimed at eliminating causes and consequences.
Symptoms
The external manifestations of vulgar ichthyosis should include symptoms such as uneven skin peeling, thickening of its upper layer, changing tactile sensations on altered skin sections. The mechanism for activating the disease is as follows:
- excess of keratin production, which is accompanied by a change in skin structure;
- an increase in the speed of moving keratinocytes to the top layer of the epidermis;
- slowing down the rejection of dead cells by strengthening the links between them in the accumulation of decay products in the tissues of cells;
- the acquisition of dystrophic epithelium cells, the appearance of bubbles (vacuoles) in the upper layer of the skin, change the thickness of the epidermis.
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- With the development of vulgar ichthyosis, hair loss occurs, and the peeling wears a flour-shaped, seven-shaped or fine-plane character. Also, the fragility and thinning of the nail plates are often observed, and the lesion of the skin takes place throughout the body, the exception is only the side surfaces, the berical and cervical regions.
- Schek coloring may vary: from gray to whitish and black. The front surface of the lower leg is covered with scales in the form of fish scales and has a shiny surface.
Symptoms of ichthyosis are usually stronger in winter when the air is colder and land.
Symptoms of ichthyosis include:
- sadness of the scalp
- skin itch
- scale education on the skin
- very dry skin
- thickened leather
- rarely: thinning of nails, bones, hair and teeth, conjunctivitis, allergy
The symptoms of ichthyosis is difficult not to notice - with ordinary (vulgar) ichthyosis, which is most often diagnosed, the skin first becomes dry and rough, after which the skin during ichthyosis is covered with small whims or gray-black flakes, which is firmly connected to each other like fishing schea.
At the same time, the elbow and populated bends, the armpits and the area in the groin, are not amazed during ichthyosis. In severe cases of ichthyosis, deep, painful cracks on the soles of the legs or palms can develop.
See what ichthyosis looks in the photo, you see, all manifestations are very symptomatic.
The initial signs of ichthyosis may be dry and rough skin.
The clinical picture of ichthyosis is largely determined by the form of the disease. In our article, we will consider the symptoms of the most common variety of this ailment - ordinary (or vulgar) ichthyosis.
It is very often amazed by a moral part of the head, the hair is thinned, fall out, can come full baldness. The flakes are larger and dense, in contrast to vulgar shape, have a darker shade.
Patients suffer from organ impairments. Violations may develop bone system and mental development, the cornea of \u200b\u200bthe eye is muttered, often cases of epilepsy.
Congenital ichthyosis in children is manifested in a number of violations and malformations. Such children are born prematurely, with a small weight. The skin of the child is completely changed and reminiscent of a shell, which gives cracks, and blood oozing.
At the time when the development of the skin in the child is broken, the rest of the organs, tissues and mucous membranes continue to develop normally. The skin is not elastic, it holds and turns the attached fabrics outward. The face and the whole body of the child are very deformed and accept an unnatural look.
The cause of high mortality in newborns is due to the fact that the burdown tissue grow up and pulls or completely scoring vital organs.
The newborn can not breathe and eat, internal organs Have defects in development, infection is joined. Such children are also called "harlequins", and the disease is the "symptom of harlequin".
Ichthyosis in children can affect all skin chimples or only certain sections. In such places, the skin bluses, swelling seals are tested under it, especially in places where skin folds are located.
Pathological sites on the face can disfigure appearance, turning the eyelids or the area of \u200b\u200bthe mouth and the nose. But there are chances for survival in such children.
The skin disease under consideration affects the top layer of the epidermis, the process of keratinization of young cells is disturbed, which is manifested in skin change:
- on the surface of the skin, especially in the area of \u200b\u200bthe neck and face, as well as on the chest, there are areas with small dry flakes between the blades, which over time change bodies from the color of healthy skin to grayish and dark gray;
- the whole body of a child with a given disease is covered with a thin film having a yellowish brown shade that can transform and take the kind of smallest scales;
- the skin acquires unhealthy appearance, affected areas with a nonsense form of disease localized. The exclusion of the lesion is deeply folds of the skin, the side surfaces of the body. However, with the course of the disease, it is manifested even in those areas that are usually not subject to change;
- the scales in the activation of the disease can acquire the type of shields that give the skin a kind of snake;
- the defeat of the nail plates during lamellar ichthyosis is infrequently manifested, but sometimes it can be observed by the appearance of strips of red or white colors on the nails, the edge of the nail is thinned and starts crumbbed.
To remove the listed symptoms required timely diagnosiswhich allows you to draw up the correct treatment and removal scheme of unpleasant manifestations.
The first sign of the development of ichthyosis becomes dry skin. The skin becomes crash.
Over time, it is compacted and cracks, and white or gray crusts appear on its surface. On the surface of the palms and feet, the skin pattern becomes more pronounced.
Nails in the amazed areas are thin and weak, easily break. The hair is almost not growing, and if they are still growing, then very weak and rare.
Signs of the disease affect the upper layer of the skin, namely, the keratinization of new cells of the epidermis is disturbed.
The process manifests itself in the following changes:
- Zones are formed with small scaly plates, which are gray with time. Changes most often occur on face and neck, chest, between the blades.
- The body of the child is covered with a brown-yellow tint film that can become scaly.
- With medium-hard form, the affected areas are more localized and rarely affect the folds of the body.
- With the active development of the disease, flakes can acquire the species of plaques and becomes like snakescales.
- The changes of the nail plate is very rare, but when they are manifest, there is an appearance of red and white strips, and the free edge of the nail becomes brittle.
- Eyelids and lips in children turn out.
- When the skin spread to the folds, cracks are formed in these places, which constantly remain wet, increases the risk of infection.
Manifestations of such characteristic symptoms Requires differential diagnosis, since there are several types of ichthyosis and the definition of the type of disease is important when drawing up the correct treatment regimen.
Diagnostics
Ordinary ichthyosis can be diagnosed already with an external inspection of the skin of the patient with complaints of increased dry dryness, thickening it, hair loss.
The most characteristic manifestation of vulgar ichthyosis is the defeat of the mouth of the pollylicle of the hair with the masses of the horny epithelium, while the bore or reddish-gray buccorkas are formed, covered with scales of the skin and occasionally surrounded by a reddish wedge.
However, the formulation of accurate diagnosis is hampered by the great dispersion of external inspection. Therefore, for more accurate diagnosis to conduct a number of additional studies, which, however, is very expensive.
Their small availability also makes it difficult to diagnose the disease and clarifying its variety. Therefore, much attention in the presence of characteristic manifestations is given to the following actions of the diagnostic nature:.
A dermatologist specializing in skin disorders can usually diagnose ichthyosis visually.
Your doctor will ask you about any family history of skin diseases, age when you first experienced the symptoms of ichthyosis, and whether you suffer from any other skin diseases.
Your doctor will start to fix where dry skin spots appear. This will help the doctor track the effectiveness of the treatment of symptoms of ichthyosis.
The dermatologist can also perform other tests, such as blood test or leather biopsy to eliminate other skin diseases, such as psoriasis, which causes similar symptoms. Skin biopsy includes the removal of a small area of \u200b\u200baffected skin for research under a microscope.
Due to the high cost and increasingness of specific research, the main importance in the diagnosis has anamnous data and characteristic clinical symptoms. At the same time, the greatest attention is given:
In most cases, the diagnosis of ichthyosis does not cause difficulties. To hold a doctor, a dermatologist is enough to inspect the patient and perform a histological study of the skin sample.
With suspected congenital forms of ichthyosis or ichthyosis, the harlequin pregnant woman shows the tests of amniotic fluid and a fetal skin sample (it is performed on the 19-21 week of pregnancy). When confirming the diagnosis of "Frup Harlequin", a pregnancy interruption is shown.
In order to diagnose the disease, in most cases there are enough clinical manifestations, in connection with which you can also assign treatment. Congenital ichthyosis on external signs can be confused with eryermodermy or other pathology.
Put a histological examination in diagnostics will help.
With the help of ultrasound, you can diagnose congenital ichthyosis, starting from 16-20 weeks of pregnancy. In this case, doctors recommend to make an abortion.
With other forms of ichthyosis, skin peeling can begin in a few weeks after birth. Usually for the diagnosis of a sufficient physical examination, sometimes a biopsy of the skin can be carried out.
Since the external manifestations of lamellar ichthyosis are rather noticeable, even the overall inspection of the affected parts of the body makes it possible to obtain a preliminary idea of \u200b\u200bthe existing disease. However, the refined diagnosis can be delivered only when additional laboratory studies are carried out.
Active sweating with a given disease is characteristic of those areas that are amazed: more dense and red leather has a disturbed process of thermoregulation, which leads to sweating. The skin is stretched, actively peels off, which can be a sign of lamellar ichthyosis during diagnosis.
The formulation of such a diagnosis as ichthyosis requires a compulsory visit to the dermatologist. The doctor already on the basis of a visual inspection may be diagnosed. To refine the patient's condition, such diagnostic methods can be used:
- histological analysis of skin tissues;
- the biopsy of the fetal dermis between 19 and 21 weeks of pregnancy.
Treatment
Reducing the manifestations of ichthyosis and improved skin condition allows you to correct the patient's condition, but it should be aware that there are no special treatment methods for full cure from this disease.
The treatment of the disease consists in the use of vitamin A, which improves both the overall condition of the skin, and ensures normalization of the epidermis energization process.
Therapeutic method
In parallel with medicinal treatments, the following recommendations should be followed:
Researchers in medicine have not yet found a medicine from ichthyosis, today treatment of ichthyosis is reduced to the control of your symptoms and facilitating the condition for the disease.
Specific drugs and methods of ichthyosis therapy does not exist. The basic principles of treatment are to use vitamin derivatives "a".
For these purposes, a reception is assigned inside the palmitate retinol in a daily dose of 3.5-6 thousand units per 1 kg of body weight. The duration of the treatment course is about 2 months, followed by the transition to supporting doses.
Intervals between treatment courses - 3-4 months.
The complex therapy also includes drugs with a zinc content, which must be taken by courses with a duration of at least 3 months - "zincter", "zincite", "zinc". At the same time, other vitamins are prescribed - mainly vitamins "C", "E" and group "B".
Need careful I. correct care Skin Covers using external moisturizing and vitamin means. Recommended moisturizing creams during ichthyosis should necessarily contain derivatives of vitamin "a".
Treatment at home is in the daily reception of the baths. The water temperature should be about 38 ° C.
How to wash with ichthyosis? You can not use soap and take a cool shower. Soap can sometimes use only for processing areas with a dye.
It is best to use special body gels with natural oils and extracts medicinal plants, White (desonyless) naphthalan, emulium-triac emulsion for swimming.
In the bath you can add a starch solution, a saline solution. Also applied phytotherapy in the form of chamomile infuses, calendulas, sage, turn, brave of birch kidneys added to the bath when bathing.
After receiving the bath for a better branch of the scales, it is necessary to rub a cream with vitamin "A" with the addition of salicylic acid (1%) and a polyvitamin salt (0.25%), born-salicyl ointment (2%), a lotion with urea content, "urms" (ointment with urea), "solkocherassal" (ointment with urea and salicylic acid), etc.
Genetic forms of ichthyosis are not amenable to complete cure, and with the forms of this dermatological disease, the therapy of the main illness, which caused this pathology is shown the patient.
Currently, studies are underway to create drugs that would be able to influence the changed genes and contribute to their recovery.
It is possible that their creation will allow patients with hereditary forms of ichthyosis to get rid of this unpleasant and severe disease.
Treating all forms of ichthyosis is carried out by a dermatologist. The need to hospitalize the patient is determined by the severity of the clinical case.
The following drugs can be assigned to patients with various forms of hereditary ichthyosis:
Folk healers give a lot of advice for those who suffer from scaly leather. All of them are different and aimed at at least somehow facilitate the course of the disease and reduce discomfort.
However, remember, follow the advice of people who have a dubious attitude to medicine, should be careful. If the grandmother on the market will be annoyed to put the drug "from the sore of Ichthyosis of Claiman", which in nature does not exist, then it is better to stay away from such an "Intern" away.
But in folk medicine there are really standing recommendations. Basically, these are teas and baths on herbs decoctions.
There are also homemade ointment recipes that are aimed at moisturizing, disinfection and skin food. All of them consist of natural ingredients, and make their homes easily.
Perhaps you will pick up a few efficient and simple recipes.
Above the search for medicines for people suffering from genetic diseases, genetics and laboratories of many countries are working. It remains to hope that Panacea will soon be found, and we will be able to ever save humanity from such an unpleasant and even terrible disease.
Trikhophysu - Causes and Signs, Symptoms and Treatment
Specific treatment of ichthyosis does not exist, only treatment symptomatic manifestations: Maintaining the respiratory function, stabilization of the hormonal background. Special attention is paid to skin care complexes:
Reception of a warm bath every day with rubbing leather with fatty creams, vaseline;
Reception of retinol (vitamin A) inside and rubbing into the skin;
Injections of cyanocobalamin;
Use of creams with 10-15% urea content;
With severe ichthyosis, hormonal ointments are used.
The disease is chronic, lasts all his life and even progresses. Symptoms are weakening or disappearing at all in a warm and wet climate, so air humidifiers greatly facilitate the patient's condition.
Hytyosis Harlequin photo
It should be known that there is no complete cure of this disease; However, modern drugs and an integrated approach make it possible to largely improve the overall condition of the patient and restore skin health.
After diagnostic studies, the entire course of treatment is carried out only under the supervision of a dermatologist.
The severity of the disease determines the treatment approach; With severe and launched forms of disease, treatment is recommended in the hospital. Usually, the course of treatment includes the reception of vitamin A and its derivatives, which have a positive effect on the skin condition.
Vitamin A is usually prescribed with all forms of ichthyosis, and its quantity depends on the severity of the disease. Its long reception ranges from 3 to 5 weeks. Then follows the weekly break, after which the reception can be resumed depending on the skin condition.
Even after the resulting treatment of red skin on the skin can remain for a long time, in some cases - for life.
Medication
For external use with pronounced lamellar ichthyosis, the means eliminating the increased dryness of the skin and peeling can be applied. It can be vaseline, cream for outdoor use Dematop.
They are applied to peeled areas of affected skin and left before absorption. An increase in skin elasticity, reducing the development of active substances in the epidermis and skin saturation of lipids - the main manifestations of the relief of the listed funds.
Corrective hormonal measures of events make it possible to improve the condition of affected skin. Since often, with a given disease, a change in the functioning of the thyroid gland is observed, hormonal therapy can be assigned to the endocrinologist to normalize the work of the thyroid. These drugs include:
- Thyreokomb;
- Thyroid;
- Thyroidine.
With insufficient activity of the pancreas, the use of insulin can be assigned. However, the treatment scheme is individual and is appointed by an endocrinologist.
Treatment is aimed at relieving the symptoms and the restoration of normal synthesis of keratin in the body. For this purpose, these drugs are used:
The lamellar ichthyosis is an incurable disease, but modern methods of therapy are able to reduce the severity of characteristic symptoms and significantly reduce the frequency of recurrences.
The complex of treatment from lamellar ichthyosis may include such items:
- Vitaminotherapy: A (up to 30 drops twice a day), E, \u200b\u200bRR, C, group of vitamins B (in the composition of vitamin complexes). For long courses for 2-3 months, after the break, repeat (total the course is repeated 2-3 times a year). Etricitat, Tigays, Atriceketin (active substance of drugs - Vitamin A derivatives) are prescribed: 0.5 mg / kg body weight of the child.
- Hormonotherapy. The corticosteroid group is prescribed with highly pronounced symptoms and diagnosed failures in the work of the humoral system. Correcting drugs are appointed by a doctor taking into account the age of the child and the general state of health. The treatment and dosage diagram must be strictly observed.
- Keratolithic. Applied water solution Propylene glycol - applied to the skin and closes with a plastic film overnight. After the course of treatment, the duration of which the doctor defines, supporting procedures are carried out as needed (1 time in 7 days, month).
- Skin Care: Moisturizing Means with Vitamin A. For older children and adolescents, the use of urea and salicylic acid preparations is possible - for softening the burned particles. Tools are used: Losterine, Topicrem, Modify, Dermatol, Emolyum, La Cree, Sleel (Lanolin), 1% White Mercury Ointment, Salcossurian or 1% Salicylic Acid Ointment, Vaseline, Almond Oil.
- Physiosters: balneotherapy, mud, ultraviolet irradiation (course up to 30 sessions in a row with a break for a month).
Attention! Therapeutic measures are able to ease the life to the patient, but full cure Disease is impossible.
Often ichthyosis proceeds with accompanying infections - in this case, the course of antibiotics is used.
Lamellar ichthyosis is a disease that requires constant therapy. At the birth of a child with a severe form of this disease, the percentage of survival is about 20%, but at the late manifestation, pathology is extremely rarely becomes a threat to life.
Complications
In the absence of treatment or improper selection of the therapy method, probably worsening the manifestations of the disease, which significantly worsens the overall quality of the patient's life.
The appearance of skin edema, the weakening of its functions, and in infancy, with the active development of complications, the fatal outcome is likely.
To complications of lamellar ichthyosis include the transition of the initial form in a more neglected when the symptoms are expressed as much as much as possible and cause discomfort to the patient. Improving mortality of babies if they have little disease, it is insignificant.
Sources
- https://dermhelp.ru/disease/ihtioz.
- http://heal-skin.com/Bolezni/other/IXTIOZ-KOZHI.html.
- http://badacne.ru/kozhnye-zabolevaniya/ihtioz-kozhi-foto.html
