Ichthyosis and ichthyosiform dermatosis

This is also a heterogeneous group of hereditary keratinizing diseases, differing in the appearance of symptoms, the method of inheritance, and also the attendant symptoms. A common symptom of these diseases is dry skin and pronounced stratification of scales.

There are two forms of ichthyosis - normal and X-linked. Ichthyosiform dermatoses are a more diverse group of keratinizing diseases, including diseases such as non-bulbous ichthyosiform erythroderma, lamellar ichthyosis, bullous ichthyosiform erythroderma, and a number of syndromes.

Ordinary ichthyosis is the most common, its frequency is estimated as 1: 3000. This pathology is inherited by an autosomal dominant type. The first signs of the disease appear already in early childhood, usually after 3 months of age. At the same time, both sexes are affected equally often. Ichthyosis is manifested by dryness and scaling of the skin, most pronounced on the inner surface of the limbs. Skin folds with ichthyosis are not affected. The palms and soles are dry, with a pronounced cutaneous pattern and deep folds, which gives them a senile appearance. Nails are not changed. Many people with these symptoms have signs of allergic reactions. With age, the intensity of the process subsides somewhat.

X-linked( sex-linked) ichthyosis is inherited by a recessive type, which explains its more rare occurrence, with a frequency of 1: 6000 in males. The full development of all the symptoms characteristic of this form of ichthyosis is observed only in boys. Unlike ordinary ichthyosis, this form of the disease can manifest itself from birth. Scales formed on the baby's skin, larger, reddish color. The skin folds, neck, back surface of the feet are also involved in the process, but there are no changes on the palms and soles. Skin changes are more pronounced on the inner surface of the extremities and on the abdomen, peeling can be significant on the scalp. Often, an additional examination reveals the opacity of the cornea, as well as the underdevelopment of the genitals.

Ichthyosiform erythroderms differ from usual ichthyosis with the presence of all the symptoms already at the time of the birth of the child, as well as the presence of signs of inflammation( swelling, redness, pain, local skin temperature increase).With the bullous form on the skin there are blisters, especially in the newborn period and early childhood. In addition, the skin folds are also affected, quite often - the appendages of the skin( hair, nails).

Bullous ichthyosiform erythroderma is characterized by pronounced stratifications of scales on the inner surface of the joints, presence of blisters, accelerated growth of hair and nails. Changes in the skin on the face are slightly expressed, located mainly in the lower part of it. The disease is inherited by an autosomal dominant type.

A special place is occupied by the so-called congenital ichthyosis, which is a diverse group of skin diseases, which, depending on the severity of the process, may be incompatible with life( harlequin fruit, or congenital heavy ichthyosis), degenerate into one of the forms of ichthyosiform erythroderma( the so-called collodionfetus) or almost completely spontaneously heal in the first months of a child's life.

Intermediate space between common and focal keratoses occupy figurative variable erythro-keratoderma, transmitted by autosomal dominant type of inheritance. The main feature of these conditions is the presence of skin rashes, which have contours in the form of various figures, changing the boundaries within a few minutes, hours or days.

Treatment of .The main tool used to treat ichthyosis is vitamin A and keratolytic drugs. In ichthyosiform erythroderms, a drug such as tigazone( at an initial dose of 0.5-1 mg / kg of body weight, in the presence of blisters - 0.3 mg / kg of body weight per day) is most effective. Prenatal diagnosis is possible with some forms of the diseases of this group. For this purpose, a specialized study of the amniotic fluid is necessary. Prenatal diagnosis of congenital ichthyosiform erythrodermia is carried out with the help of microscopic examination of the fetal skin, taken on the 18-20th week of pregnancy.