constitute a heterogeneous (diverse) group of diseases characterized by
excessive peeling of the skin. The term "ichthyosis" comes from the Greek «ichthy», which means "fish". The most common hereditary
forms of ichthyosis, but it is possible the occurrence of this condition is secondary
in relation to other diseases.
This is the most common form of hereditary diseases
cornification (1: 3,000 - 1: 4,500 of the population). Cause of the disease is unknown.
There are several clinical forms due to different groups
mutant genes, the biochemical defect that is not completely
decrypted. Attaches great importance to vitamin A deficiency,
endocrinopathies (disruption of the endocrine gland - hypothyroidism, gonads).
Average (vulgar) ichthyosis - The most common form.
Inherited in an autosomal dominant manner (inheritance is passed on to children
parents with congenital form
disease). Developed in the first 2-3
years of life and is characterized by sudden dryness of the skin, formation
scales on the surface whitish or grayish color, heavy
cases takes the form of brown plates and coarse boards, solid
to the touch. Leather large skin folds and folds remains unaffected.
On the face peeling is usually negligible, on the palms and soles
underlined drawing skin lines. Sweating is reduced, it is possible
dystrophy of the nail plate and hair. Ordinary ichthyosis often
combined with atopic dermatitis, seborrheic dermatitis, bronchial
asthma. the skin condition improves with age, in the summer and
humidity worsens in cold dry weather.
abortifacient ichthyosis - Kind of easy flowing
ordinary ichthyosis, characterized by dryness and skin roughness
with the presence of small scaly scales on the extensor surfaces
limbs and buttocks.
White ichthyosis characterized in white flakes.
echinated - The presence of warty horny layers in the form of pointed spikes and needles.
Simple (pityriasis, pitiriaziformny) ichthyosis - Formation of small (0.5 cm) grayish scales attached to the skin only in its central portion.
serpentine ichthyosis - The presence of thick flakes of dirty gray, tightly adjacent to each other like flaps (reminiscent of snake skin).
Black ichthyosis characterized by a brownish-black scales, firmly seated on the skin.
Brilliant ichthyosis - Flakes of transparency and their localization is mainly on the limbs.
X-linked ichthyosis usually develops due
sulfatase deficiency of placental steroids. Unlike
ordinary ichthyosis can exist from birth. Full clinical
picture was observed only in boys. The flakes are larger
dimensions than ordinary ichthyosis, brownish color. Defeat
more extensive in the process may involve the skin folds, neck, rear
foot, limb flexor surface, at least in the back,
scalp. More intensively than in the ordinary
ichthyosis, affected the stomach. Not affected skin of the palms and soles. Frequently observed turbidity
cornea, hypogonadism (hypoplasia of the genitals), cryptorchidism (absence in the scrotum one or both testicles). There may be various defects
development (microcephaly, pyloric stenosis, skeletal anomalies)
ichthyosiform erythroderma (Congenital ichthyosiform
erythroderma Brock). Unlike conventional clinical ichthyosis is
the existence of inflammation at birth.
Ichthyosiform erythroderma bullosa (hyperkeratosis
epidermolitichesky) - blistering and the presence of symptoms
Nikolsky (epidermis bundle with formation of bubbles: the skin becomes
similar to tissue paper).
Erythroderma congenital ichthyosiform nebulleznaya - education
large scales to be tightly fixed to the skin (especially in
area of skin folds), accelerated hair and nail growth, hyperhidrosis
palms and soles.
Congenital - Hereditary ichthyosis occurring during fetal development and is found in newborn.
Acquired ichthyosis. The development of dry, flaky skin is not
necessarily due to hereditary reasons. This is the case with
malignant tumors, eating disorders, metabolic disorders,
drug therapy. In malignant tumors observed
reduction of dermal synthesis of lipid malabsorption (Malabsorption syndrome - a disorder characterized
absorption in the small intestine of one or more nutrients and appearance
metabolic disorders), immune
violations. Many eating disorders accompanied by pathological
changes in metabolism of vitamin A. Chronic kidney
deficiency results in hypervitaminosis A - this causes coarsening
and peeling of the skin. Hypovitaminosis A cause of follicular hyperkeratosis
and dry skin. Mechanisms of development of medicinal ichthyosis different.
Prenatal diagnosis of congenital ichthyosis held with
the relevant family history. Fetal skin biopsy conducted
between 19 minutes and 21 weeks can detect thickening of the stratum corneum, which is not
observed in the rate of up to 24 weeks. This corresponds to a plate
ichthyosis, hyperkeratosis epidermolitichesky, "Harlequin fetus."
The treatment of ichthyosis
- virtually all forms of ichthyosis - vitamin A in courses for 2-3 months, 2-3 times a year or tigazon (etretinate);
- in dry ichthyosiform erythroderma and lamellar ichthyosis -
etretinate, isotretinoin 0.5 mg / kg. Retinoids are contraindicated in
- with bullous form - cloxacillin or erythromycin;
- keratolytic agents: 6% -s salicylic ointment, 5-10% -s urea ointment.
The course of acquired ichthyosis is usually facilitated by the treatment
underlying disease. The therapy is complex congenital ichthyosis, especially its