Ichthyosis

Ichthyosis is a hereditary disease manifested by a generalized disturbance of the keratinization of the skin.

The etiology is unknown. There are several clinical forms caused by different groups of mutant genes, whose biochemical defect is not deciphered finally. They attach great importance to vitamin A deficiency, endocrinopathies (hypothyroidism of the thyroid gland, sexual glands).

Symptoms, course. Ordinary (vulgar) ichthyosis is the most common form. It is inherited by an autos-but-dominant type. It appears in early childhood and is characterized by dry skin, the formation of scales on its surface whitish or grayish color, in severe cases acquiring the appearance of brown plates and coarse shields, dense to the touch. The skin of large skin folds and folds remains unaffected. Facial peeling is usually insignificant, on the palms and soles of the skin lines are emphasized. Sweating is reduced, dystrophy of nail plates and hair is possible. Ordinary ichthyosis is often combined with atopic dermatitis, seborrheic eczema, bronchial asthma.

Congenital ichthyosis is expressed already at the birth of a child, is divided into ichthyosis of the fetus and ichthyosiform erythroderma. Ichthyosis of the fetus is rare, inherited by autosomal recessive type, develops during the III-V month of pregnancy. At birth, the child's skin is covered with powerful horny layers, reminiscent of a tortoise shell or a crocodile skin. The oral opening is stretched, slightly moving, or, conversely, sharply narrowed. Children are often premature and unviable. With ichthyosiform erythroderma, the skin at birth is covered with a thin, dry yellowish film resembling a collodion, after the rejection of which there is a persistent diffuse hyperemia of the entire skin (including skin folds) and lamellar ecdysis, the degree of which increases with age, and erythroderma weakens. There is also a bullous variety of congenital ichthyosiform erythroderma - epidermolytic ichthyosis, which is characterized by a more severe course with the formation of blisters. With congenital ichthyosiform erythroderma, eye damage (ectropion, blepharitis), nail dystrophy, hair, keratosis of the palms and soles, lesions of the nervous and endocrine system are possible. The disease lasts a lifetime.

Differential diagnosis is most difficult in childhood. With congenital ichthyosiform erythroderma it is carried out with desquamative erythroderma of newborn Leiner, bullous epidermolysis, psorinatic erythroderma.

Treatment. Vitamin A inside up to 30 drops 2 times a day for a long time with repeated courses, Aevit in / m or in capsules, tigazone, vitamins B6 and B12 in / m, preparations of iron, arsenic, thyroidin (under the control of thyroid function). In severe cases of ichthyosiform erythrodermia, corticosteroid preparations are given orally. Also shown are ultraviolet irradiation, sea bathing, baths with the addition of borax, soda, starch, sea salt. Outer: fatty creams with the addition of vitamin A, 10% cream with sodium chloride, urea, pork fat.