Ichthyosis means a violation of keratinization of skin tissues. It is manifested by specific formations, which, with all their appearance, are very similar to fish scales. It proceeds as a dermatosis and can be inherited.
The disease may appear over time or be congenital (Harlequin ichthyosis). The exact causes of its occurrence have not yet been established. It can be said for sure that it develops due to a gene mutation. Excessive amounts of amino acids in the blood lead to the disruption of protein metabolism, and due to excess cholesterol, the metabolism of fats is disrupted. Such manifestations are signs of a genetic malfunction that can lead to ichthyosis.
Thermoregulation of patients is impaired. Due to the excessive activity of enzymes, the processes of skin respiration are accelerated, problems arise with the thyroid gland, adrenal glands, and sex glands. Almost always, one can observe a deficiency of keratin, which has deviations in the structure.
With ichthyosis, dead cells are rejected extremely slowly. For this reason, keratinous scales begin to appear on the skin. Over time, amino acid complexes begin to accumulate between them. The skin hardens from this. The formations are attached to each other, it hurts to remove them.
Ichthyosis Harlequin
This form of the disease is the most severe. The fact is that Harlequin ichthyosis develops in the fetus, which is located in the womb. This disease is inherited in an autosomal recessive manner. Its first manifestations can be observed after the sixteenth week of fetal development.
Harlequin ichthyosis leads to the fact that the skin in a newborn becomes like a shell. This "shell" consists of a kind of dark shields. Their thickness can reach one centimeter. They themselves are pointed, flat, dense.
Cracks can be seen between the shields. Harlequin ichthyosis affects the entire surface of the body and even the face. The forecast in this case is extremely unfavorable. Children die early. This is due to the fact that a terrible disease affects all organs. Of course, the existence of this becomes impossible. This ichthyosis is also caused by genetic mutations. In children, all body processes are disrupted, cholesterol rises. There is practically no immunity in those born with this disease, sweat glands work extremely poorly.
Vulgar ichthyosis
This species is much more common than others. It is inherited. Its first signs can be observed in the third month of the child's life. In some cases, symptoms begin to manifest much later.
With it, the surface of the skin dries, becomes rough and becomes covered with scales. The face, as a rule, is not affected. Patients have problems with the kidneys, cardiovascular system, and liver.
X-linked recessive ichthyosis
It appears only in boys. The first symptoms can be observed in the second week of life. This species is characterized by brown-black scales on the skin. The skin becomes like a snake or a crocodile. The disease can be accompanied by epilepsy, can lead to mental retardation.
Ichthyosis: treatment
It all depends on the severity of the disease itself. Treatment of ichthyosis can be carried out in a hospital or on an outpatient basis. A very long vitamin course is prescribed for patients. Lipotropic drugs are also prescribed - this helps to avoid hardening of the scales. Measures are also being taken to restore immunity.
Hormone therapy is sometimes used. Local treatment includes the treatment of scales with potassium permanganate, baby cream and so on.
The disease progresses very quickly, which means that delay will lead to the fact that internal organs will have to be treated.