Encyclopedia

Ichthyosis

Ichthyosis is a hereditary skin disease occurring as a type of dermatosis. It is characterized by a diffuse disorder of keratinization and manifests itself in the form of scales on the skin, resembling fish scales.

What are the symptoms?

The common or vulgar form of the disease appears before the age of 3, but in most cases the diagnosis is made by the third month of life.

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This is the most common form of the clinical condition, which is inherited in an autosomal dominant manner.

Initially, the skin becomes dry and rough, then becomes covered with small whitish or gray-black scales that adhere to each other.

The disease does not affect the area of the elbows, popliteal fossa, armpits and groin.

Mucous peeling of the skin and callousing appear on the palms. The severity of the disease depends on how deep the gene mutation is.

An abortive course is possible, when the only manifestation is dryness and not very strong peeling of the skin in the areas around the joints.

In the disease, the teeth, hair and nails undergo dystrophic changes. Dry brittle hair, peeling and easily brittle nails and the appearance of numerous caries are characteristic.

Quite often the clinical condition is accompanied by eye diseases, most often chronic conjunctivitis and retinitis. Affected people have a family history of myopia, which begins to manifest itself in childhood.

Because immunity is weakened, allergic reactions and purulent infections are constant.

Subsequently, disorders in the functions of the internal organs are observed, the most often indicated are cardiovascular insufficiency and liver disease.

The recessive form occurs only in males, although it is inherited from the X chromosomes and is distinguished by the fact that the cause of the disease is a defect in enzymes in the placenta.

Clinical manifestations of ichthyosis appear in the second week of life and less often immediately after birth.

The stratum corneum of the skin appears as large, dense red to brown scales and look like shields.

The skin between the scales is cracked and looks like that of crocodiles or snakes. Affected children often have mental retardation, skeletal abnormalities, and epilepsy.

Juvenile cataracts and hypogonadism occur in 10-12% of cases.

The congenital form of the disease develops intrauterinely in the 4-5th month of pregnancy. At birth, the baby’s skin is covered with thick horn shields of gray-black color.

And the thickness of the scales can reach 1 cm, they have a different shape, most often smooth or jagged, between them the skin is also cracked.

Due to the excessive density of the scales and their tight adherence, it may be too wide open or so tightly closed that the feeding tube is difficult to pass.

Treatment of ichthyosis

Treatment is carried out by a dermatologist on an outpatient basis, and if the disease is severe, hospitalization is recommended.

Vitamins of group A, E, B, vitamin C and niacin are prescribed in high doses with long, repeated courses. Medicines that have a lipotropic effect soften skin scales.

These are the preparations whose active ingredient are vitamin U, lipamide.

Transfusion of blood plasma, gamma globulins, preparations containing iron and calcium, and also those with aloe extract have been found to be effective in stimulating immunity.

Thyroidinum is used for thyroid gland damage and the related development of hypothyroidism, and insulin for hypofunction of the pancreas.

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