Contents
What is ichthyosis?
Ichthyosis is a skin disease that has much in common with dermatosis. Pathology is acquired by heredity and is characterized by a uniform violation of keratinization and the appearance of fish-like scales on the surface of the body.
Of all congenital skin diseases, ichthyosis accounts for about 87% of pathologies. In this case, mutations occur in various genes of the epithelium, which leads to a change in its structure. Symptoms of the disease can be very diverse. However, regardless of the form of ichthyosis, there is a single sign of the disease – increased keratinization and peeling of the upper layer of the dermis.
Despite the high development of medicine, the treatment of ichthyosis is a difficult task that confronts the doctor. Although scientists have already been able to develop therapeutic schemes to improve the quality of life of such patients. Infants can also receive treatment.
Ichthyosis in adults develops very rarely. This happens because of other serious illnesses. The treatment regimens for adults do not differ from the treatment regimens for children.
Causes of ichthyosis
Causes of congenital ichthyosis
Gene mutations are the cause of all congenital ichthyosis. Protein compounds, enzymes and other substances that take part in the formation of the dermis are produced with certain disorders.
Until now, the exact causes of gene abnormalities are unknown, but scientists have identified some risk factors for the development of ichthyosis, including:
hereditary predisposition. The likelihood of developing ichthyosis is higher in the presence of such pathologies in a family history as: Down syndrome, Patau syndrome, Shershevsky-Turner syndrome, etc.
Bad habits in parents, for example, drinking alcohol by a pregnant woman, smoking, substance abuse, drug use.
Chronic illness in parents. Various infectious processes are important, for example, pyelonephritis, hepatitis, gastritis, etc.
Impact on the body of harmful factors: radiation, polluted air, heavy metals, etc.
All the harmful factors that affect parents can affect the health of the child and lead to the development of ichthyosis.
Causes of acquired ichthyosis
Sometimes ichthyosis develops during life.
The reasons may be as follows:
Deficiency in the body of vitamin A.
Malignant tumors with the development of paraneoplastic syndrome. The patient’s immune system begins to produce immune complexes against their own skin and destroy it.
Ichthyosis on the background of alcoholism. Blood vessels are destroyed, the skin does not receive normal nutrition, which causes the development of the disease.
Ichthyosis, which develops throughout life, does not pose a threat to human health. Its symptoms are not as pronounced as in congenital forms of the disease. The skin of such people is prone to increased flaking, the skin pattern will be enhanced in the area of the feet and palms. You can cope with the disease if you identify the cause of its occurrence. When etiotropic treatment does not achieve positive results, therapy is carried out according to the same scheme as in congenital forms of the disease.
Forms of congenital ichthyosis
Depending on the timing of the onset of the disease and the place of mutation, there are such types of ichthyosis as:
Ichthyosis in newborns. Mutations occur on the female sex chromosomes. The disease begins to develop even during the period when the child is in the womb. He will be born with symptoms of the disease. Male babies are most often affected, and they receive the mutated gene through the female line.
These types of ichthyosis include:
Lamellar.
Erythroderma ichthyosiformis.
Erythroderma bullous ichthyosiform.
Harlequin ichthyosis.
Ichthyosis delayed in time. The mutation occurs on the non-sex chromosomes. For the first time, the disease manifests itself after 2 months – 1 year after the birth of the child. The disease has an uncomplicated course compared with ichthyosis of newborns. Ichthyosis vulgaris refers to a pathology delayed in time.
Depending on the form of the disease, the symptoms of the disorder will vary.
Symptoms of congenital ichthyosis
The child is born “packed” in a light brown film (colloidal fetus). After a few hours or days, the film comes off.
In the future, the disease can proceed according to 4 types. The symptoms are pronounced, so the diagnosis of a specific form of pathology is not difficult:
Lamellar ichthyosis:
The skin is very tight.
The eyelids and lips of the child are turned outward.
Large horn particles are separated by cracks. They are deep, get wet all the time, a transparent intercellular fluid appears from them.
The feet and palms are covered with thick scales of skin, which are separated by cracks.
Ichthyosiform erythroderma:
After birth, the baby is covered with a film that dries quickly, cracks and falls off.
The skin looks like a child has been burned. It is flaky and red in color.
The skin is tight. As the child grows older, silver stripes appear on it.
The baby’s ears are irregular. However, this symptom does not occur in all patients.
Bullous ichthyosiform erythroderma. This form of the disease manifests itself in the same way as ichthyosiform erythroderma, but, among other things, ulcers and blisters appear on the skin.
Harlequin syndrome:
This type of disease is the most difficult and life-threatening for a child. The horny layer of the skin thickens over the entire surface of the body.
The lips and eyelids of the child are turned out.
The cracks are very deep and look like wounds.
The head of the newborn is reduced in size.
As the child grows older, the symptoms of the disease become less intense (unless he has been diagnosed with harlequin syndrome). The skin becomes normal in color, but continues to remain tight and flaky all the time. If the patient develops a laminar form of pathology, then the silver stripes on it persist throughout life.
Symptoms of ichthyosis vulgaris
The disease makes itself felt 2-12 months after the birth of the child. The manifestation of pathology falls on the cold season.
Symptoms of ichthyosis can be distinguished as follows:
The skin is flaky on the body. This process does not involve the face, popliteal and axillary cavities.
On the palms and on the soles of the feet, the natural pattern is enhanced, the furrows become deep, and the tone of the dermis is weakened. This leads to the fact that the feet and hands of the child look senile.
On the hips and on the shoulders, areas of compaction appear, which will be represented by tubercles.
From time to time, the disease will worsen and subside. In the cold season, the symptoms of pathology intensify.
Treatment of ichthyosis
It is impossible to cope with the congenital form of ichthyosis forever. A person will need to constantly monitor the state of health. To do this, he will need to take medicines and make certain adjustments to his lifestyle. All forms of pathology, except for the harlequin syndrome, lend themselves well to symptomatic correction.
General therapeutic measures
If the baby is born in a film, then it is placed in an incubator. It maintains the desired level of humidity and temperature.
Immediately, the infant should receive drugs such as:
Glucocorticosteroids. There is no exact data as to why these drugs have a positive effect on the health of patients with ichthyosis. However, their effectiveness has been proven. The drugs of choice are Prednisolone or Hydrocortisone. Treatment should continue for 4-7 weeks. Then the dose of drugs is gradually reduced.
Vitamin A. It regulates metabolic processes in the skin, promotes the production of intercellular substances. The course of admission is 14 days, but at the discretion of the attending physician, it can be changed. The vitamin is sold under the name Retinol Acetate. If a child develops an allergy to it, then he is prescribed Acitretin.
Antibiotics. They are prescribed in the case when there is a high probability of joining a bacterial infection.
Electrolytes and albumin, which are administered intravenously. They improve the general well-being of the patient. For newborns, drugs are delivered into the umbilical vein.
When the disease worsens, the therapy is repeated with the use of all drugs. The doctor can remove antibacterial drugs from the scheme, or intravenous infusions, introduce other vitamins.
Local impact
It will not be possible to do without local therapy.
For this purpose, various creams and ointments are used, for example:
Uroderm with urea 2%.
Petrolatum.
Lanolin.
Atoderm, Topikrem, Foretal and other emollients. These funds do not belong to drugs, so they can be used to treat newborn children.
Dexpanthenol.
Creams and ointments with a hormonal component: Elokom, Akriderm, Belosalik, etc.
Adult patients may be prescribed drugs containing salicylic acid at a concentration of 2%. Children under 3 years of age are not prescribed.
Physiotherapy
UVI, sunbathing (no more than 40 minutes) help to reduce the symptoms of the disease. These procedures stimulate the metabolic processes of the dermis, reduce the intensity of peeling. Older people can visit the bathhouse (the air temperature should not exceed 55 ° C), go to sea resorts.
Caring for children with ichthyosis
The baby will stay in the incubator for 2-4 weeks. During this time, he receives all the necessary treatment.
After being discharged home, parents should provide the following care for the infant:
Strictly follow all medical recommendations. Treatment should continue at home. You will need to use special creams and ointments. Processing should be regular.
Monitor the condition of the child’s skin. It is impossible to ignore periods of intense peeling, it is important to eliminate all elements of inflammation in time, monitor the baby’s body temperature, etc. This will allow you not to miss periods of exacerbation of the pathology and take the necessary measures in time.
Injury to the dermis of the child is unacceptable. The skin of a baby with ichthyosis is more susceptible to trauma than the skin of a healthy child. Pathogenic microorganisms can enter through wounds. Therefore, if an injury does occur, it is necessary to treat the skin with a solution of Chlorhexidine at a concentration of 0,005%, or with another antiseptic.
It is forbidden to use hard fabrics made of wool, synthetics. This applies to underwear and bed linen. Such materials will irritate the baby’s skin, provoke itching and exacerbate the disease.
Otherwise, the life of a child will not differ from the life of healthy children. In the future, such children will become full-fledged members of society, despite their illness.
Forecast
All forms of the disease, except for the harlequin syndrome, have a favorable course. The disease does not affect the quality of life of a person if the child receives adequate therapy from early childhood. In winter, people experience exacerbations, after which the skin returns to normal. During a relapse, you need to receive adequate therapy.
Prevention of ichthyosis
To reduce the likelihood of developing ichthyosis in an infant, the following recommendations should be followed at the planning stage of pregnancy:
Parents should visit doctors, identify and treat all chronic pathologies.
A woman should be observed by a gynecologist. She will need to pass tests and get rid of all existing diseases.
If earlier children with gene diseases were born in the family, then you need to visit a geneticist and pass all the tests that he will prescribe.
6 months before the upcoming conception, you need to stop taking alcohol, drugs, tobacco.
The doctor may recommend taking folic acid, iodine supplements, etc.
Compliance with these recommendations can reduce the likelihood of developing ichthyosis in a child by 95%. During gestation, a woman should be registered with a gynecologist, undergo ultrasound and screenings. The disease is easier to prevent than to treat.
Answers to popular questions
Is ichthyosis hereditary? Yes, but it is difficult to say exactly how and when the pathology will be transmitted. If a male baby with ichthyosis was born in the family, then the likelihood of a reappearance of a baby with a similar disease is extremely high. If the pathology has developed in the delayed period, then it is difficult to make any forecast. Be sure to visit the geneticist.
Treatment depends on the form of ichthyosis? Yes. So, with harlequin syndrome, the doses of hormonal drugs are increased. The bullous form of the disease requires the appointment of antibiotics. The doctor will help determine the therapy.
During the ultrasound, the doctor revealed ichthyosis in the fetus. How to proceed? The probability of a correct diagnosis is 92-95%. The decision is up to the parents.
What are the side effects of drugs for the treatment of ichthyosis? It can be said that most medicines are safe. Hormonal agents are prescribed for a short period, so they do not have time to affect the state of the immune system.