Ichthyosis
Ichthyosis is characterized as a hereditary disease of the skin, expressed in a violation of the keratinization process. Science knows several types of this disease, some of them are incompatible with life.
Causes of ichthyosis
The main reason for the development of this disease is considered a hereditary predisposition.
Types of ichthyosis
The simplest and most common type of this ailment is considered to be vulgar (simple) ichthyosis. As a rule, it manifests itself in early infancy, but in some cases the symptoms may be hidden up to 3 years. It is expressed in dry flaky skin, especially on the legs. The skin on the face, as well as on the buttocks and in the groin folds remains normal. On the palms and soles it is unnecessarily folded.
As the child grows up, the peeling goes away, but the folding on the palms and feet often remains for life.
Doctors distinguish several types of vulgar ichthyosis:
xeroderma (expressed by dryness and slight roughness of the skin on the bends of the arms, legs, fingers);
bullous (with the formation of blisters on the skin);
horny ichthyosis (expressed by dense horny scales rising above the skin);
simple ichthyosis (with a total lesion of the skin surface);
white ichthyosis (expressed by small white scales similar to flour);
lichenoid ichthyosis (lesions of the skin resemble lichen);
brilliant ichthyosis (expressed by transparent scales arranged in a mosaic on the arms and legs);
serpentine ichthyosis (expressed by the formation of horn shields separated from each other by furrows).
Confirmation of the genetic nature of ichthyosis is its special form. This is the so-called X-linked ichthyosis. Only boys suffer from this form of the disease. The disease manifests itself at the age of several months and is expressed in the formation of large brown scales, very tightly adjacent to the skin. From the outside, such scales resemble patches of dirt stuck to the child’s skin.
There is also a rare form of fetal ichthyosis (Harlequin fruit). The disease begins to develop in the womb at 4-5 months of pregnancy. In addition to the fact that by the time of birth, the child has lesions of the skin in the form of gray-black horn plates, the baby’s arms and legs, mouth, ears and nose are deformed. The prognosis is disappointing. Children with this diagnosis are born either dead or die in the first weeks of life.
Symptoms of ichthyosis
The main symptom provoked by mutational changes in the patient’s body is a violation of keratinization of the skin due to increased keratin synthesis. It is a protein of the skin of hair and nails. With the development of ichthyosis, it becomes defective, its structure is changed.
In addition to skin disorders (dryness, peeling, itching), patients with this disease have impaired lipid and protein metabolism with the accumulation of amino acids and fats (cholesterol) in the blood.
Patients may complain of rapid overheating due to slow metabolism and impaired thermoregulation. One of the characteristic symptoms of ichthyosis is the rapid healing of small scratches and cuts lasting literally several hours. In patients with this disease, the immune system is weakened, the functions of the adrenal glands and thyroid gland suffer, and the level of sex hormones is reduced.
Diagnosis of ichthyosis
If you suspect this ailment, you should contact a dermatovenerologist. The diagnosis is made on the basis of objective data and complaints of the patient or his relatives. The diagnosis is supported by the delivery of general and biochemical blood tests. Also, a general urine analysis and skin scraping are given for histological examination.
In those cases, if one of the parents or next of kin suffers from ichthyosis, a prenatal diagnosis can be carried out. It consists in a fetal skin biopsy performed between 19 and 21 weeks of pregnancy. Fetal diagnosis will help diagnose a form such as a Harlequin fetus.
Treatment of ichthyosis
Unfortunately, ichthyosis is an incurable disease. The main goal of therapy for this disease is to reduce the severity of manifestations and reduce the frequency of exacerbations. Enhanced vitamin therapy and courses of corticosteroid hormones may be prescribed. Enhanced skin care with frequent hydration is required, since the skin is very dry with this ailment. To do this, moisturizers with vitamin A are prescribed. Adult patients are also prescribed creams with a softening effect. A very good effect for patients with such a diagnosis can be achieved by using balneotherapy methods (carbon dioxide baths, mud baths, baths with urea and starch).
