Hyperkeratosis, what is it? Types, symptoms and treatment of hyperkeratosis. Skin and venereal diseases (page 136) Retention hyperkeratosis caused by a defect

Genodermatoses are hereditary skin diseases, numbering several hundred nosological forms, manifested by various pathological processes in the skin - keratinization disorders, dyschromia and dystrophies of the skin and its appendages, nevoid and tumor processes, as well as complex disorders, including pathology of the skin and nervous system (phakomatoses), endocrine, bone and other body systems. Most common ichthyosis, keratoderma, epidermolysis bullosa. Recklinghausen's disease is a representative of phakomatoses.

ICHTHYOSIS

Ichthyosis (syn.: diffuse keratoma, sauriasis) is a hereditary disease characterized by a diffuse violation of keratinization by the type of hyperkeratosis and manifested by the formation of scales on the skin resembling fish scales. There are several forms of ichthyosis: vulgar, X-linked, fetus, ichthyosiform erythroderma.

Ichthyosis vulgaris- the most common form, accounting for 80-95% of all forms of ichthyosis. The type of inheritance is autosomal dominant. The disease usually manifests itself in the 3rd month of life or somewhat later (up to 2-3 years). Patients have functional insufficiency of the endocrine system (thyroid, sex glands) in combination with an immunodeficiency state (decrease in the activity of B- and T-cell immunity), a tendency to allergic diseases (especially atopic dermatitis) with low resistance to pyococcal and viral infections.

The clinical picture is characterized by diffuse, varying degrees of severity lesions of the skin of the trunk, limbs in the form of layers of scales of different sizes and colors (from whitish to gray-black), as a result of which the skin becomes dry, rough to the touch. The skin changes are most pronounced on the extensor surfaces of the extremities, especially in the area of the elbows and knees, while the neck and flexor surfaces of the elbow and knee joints, as well as the axillary fossae, are not affected. Follicular keratosis is also characteristic in the form of small dryish nodules with localization in the mouths of disseminated hair follicles. The skin of the face in childhood is usually not affected; in adults, peeling of the skin of the forehead and cheeks is noted. On the palms and soles, a reticulate skin pattern is expressed with changes in dermatoglyphics and slight floury peeling. The nail plates become dry, brittle, rough, deformed, the hair becomes thinner and sparse. The severity of skin changes can be different. The abortive variant of ichthyosis proceeds most easily and is characterized by dryness, slight roughness of the skin, mainly of the extensor surfaces of the extremities.

Retention hyperkeratosis caused by a defect in the synthesis of keratohyalin is revealed histologically. The proliferative activity of the epidermis is not disturbed. Clinical manifestations of ichthyosis weaken during puberty. The disease lasts a lifetime, worsening in winter. Often conjunctivitis, retinitis, pharyngitis with subatrophic lesions of the nasopharynx, otitis media, rhinosinusitis, chronic mesotympanitis.

Ichthyosis recessive X-linked isolated from ichthyosis vulgaris on the basis of genetic studies. Cases of division in the short arm of the X chromosome, X-Y translocation in the karyotype of patients were revealed; gene mutation is manifested by a biochemical defect - the absence of the steroid sulfatase enzyme in epidermal cells and leukocytes.

The clinical picture, which develops in a sweaty volume only in boys, is characterized by damage to the entire skin, including skin folds (30% of cases), in the first months of life (less often from birth). Only the palms and soles remain unaffected. In children, the skin of the scalp, face, and neck is involved in the process. With age, pathological changes in these areas weaken, and skin changes in the abdomen, chest, and extremities intensify. The scales in this form of ichthyosis are large, dark. Hyperkeratosis is especially pronounced in the area of the extensor surfaces of the elbow and knee joints. Clinically, this form is characterized by a brownish-black color of tightly-fitting scales, numerous small cracks in the stratum corneum and large (up to 1 cm) shields of a dirty gray or brown color, which makes the skin resemble a snake or lizard shell. In some cases, the clinical manifestations resemble those of ichthyosis zulgar, but follicular keratosis and changes in the skin of the palms and soles are absent.

Histologically, retention hyperkeratosis is detected (with a normal, and not thinned, unlike ichthyosis vulgaris, granular layer). The proliferation activity of the epidermis is not changed, but the production of keratohyalin (in contrast to ichthyosis vulgaris) is not impaired. Deficiency of the enzyme steroid sulfatase leads to the accumulation of cholesterol sulfate in the blood serum and the stratum corneum, increasing cell cohesion and slowing down the process of normal epidermal desquamation. In addition, cholesterol sulfate inhibits hydroxymethylglutamyl, coenzyme A-reductase, a key enzyme in epidermal steroid synthesis.

X-linked ichthyosis is also characterized by deep stromal cataract, cryptorchidism, small testicles, penis, infertility, and mental retardation are possible.

In the diagnosis of this form of ichthyosis, in addition to the clinical picture and histological data, the results of a biochemical study are of great importance, which make it possible to detect the accumulation of cholesterol in the blood serum and skin. Antenatal diagnosis of this type of ichthyosis is possible by determining the content of estrogens in the urine of pregnant women, the number of which is sharply reduced due to the absence of the arylsulfatase enzyme in the fetal placenta, which hydrolyzes estrogen precursors produced by the adrenal glands of the fetus, which can be detected by amniocentesis.

Fetal ichthyosis (Harlequin fetus)- congenital ichthyosis, developing in the embryonic period (4-5 months of pregnancy). The type of inheritance is autosomal recessive. Genetically heterogeneous, various phenotypes are manifested by the absence or presence of hyperproliferative keratins 6 and 16, profilagrin. There may be mutations that are incompatible with life - lethal mutations (on chromosome 4), which leads to miscarriage or the birth of a dead fetus.

The clinical picture of ichthyosis by the time of the birth of the child is fully formed. The skin of the newborn is dry, thickened, covered with a horny shell, consisting of gray-black horny shields up to 1 cm thick, smooth or serrated, separated by furrows and cracks. In skin folds, the lesion resembles acanthosis nigricans. Diffuse keratoderma of the palms and soles is also characteristic. The mouth opening is often stretched, inactive or, conversely, sharply narrowed, reminiscent of a trunk, barely passable for the probe. The nose and auricles are deformed, the eyelids are twisted, the limbs are ugly (club hand, contractures, syndactyly). Total alopecia and nail dystrophy are often observed, often microophthalmia, microgyria, cataracts. Most children are born dead, the rest die shortly after birth from changes in internal organs that are not compatible with life, exhaustion, sepsis.

Histologically, diffuse powerful hyperkeratosis is revealed in the epidermis - the stratum corneum is 20-30 times thicker than the entire germ layer of the epidermis and contains many lipids. The granular layer is thickened, the structure of keratohyalin granules is not changed, their number is increased, the cell membranes are thickened.

Erythroderma ichthyosiform congenita- a form of congenital ichthyosis, isolated by Brock in 1902. There are dry and bullous types. The bullous type later became more often referred to as epidermolytic hyperkeratosis (ichthyosis), and ichthyosiform non-bullous congenital erythroderma became identified by many authors with lamellar ichthyosis. However, biochemical studies and minor signs of the clinical picture reveal some differences.

Ichthyosis lamellar It manifests itself at the birth of a child with a clinical picture of the so-called colloidal fetus. The skin of the child at birth is reddened, completely covered with a thin, dry, yellowish-brown film resembling collodion. Such a film, having existed for some time, turns into large flakes. With age, erythroderma regresses, and hyperkeratosis increases. The lesion captures all skin folds, and skin changes in them are often more pronounced. The skin of the face is usually red, stretched, and flaky. The scalp is covered with abundant scales. There is increased sweating of the skin of the palms, soles, face.

Hair and nails grow rapidly (hyperdermotrophy), the nail plates become deformed and thicken; subungual hyperkeratosis, diffuse keratosis of the palms and soles are noted. A characteristic manifestation of lamellar ichthyosis is also ectropion, which is often accompanied by lagophthalmos, keratitis, and photophobia. Sometimes with lamellar ichthyosis, mental retardation is observed.

Histologically: proliferating hyperkeratosis (sometimes with parakeratosis), moderate acanthosis, hypertrophy of the papillae of the dermis, chronic inflammatory infiltrates of moderate severity in the upper layers of the dermis. Histogenesis is based on the inability of keratinocytes to form a marginal band of the stratum corneum; biochemically, an increase in the level of sterol and fatty acids in the skin flakes is detected.

Dry type of ichthyosiform erythroderma, coinciding practically in the clinical picture with lamellar ichthyosis, has the following differences: the scales are often light (with lamellar ichthyosis they are thicker, darker), erythroderma is pronounced, variable intensity (with lamellar ichthyosis is medium), there is some thinning of the hair on the head (with lamellar ichthyosis, in addition, anomalies of the hair shaft are possible), ectropion is medium (with lamellar pronounced; twisted auricles); histologically, a noticeable thickening of the epidermis with parakeratosis is detected (with lamellar also granulosis); biochemically detect an increase in the content of p-alkanes - unsaturated hydrocarbons, characterized by hydrophobicity and the ability to influence the mitotic activity of the epidermis (with lamellar ichthyosis - an increase in the level of sterol and fatty acids in the skin scales).

Epidermolytic ichthyosis (epidermolytic hyperkeratosis, erythroderma ichthyosiform bullosa)- a rare form of congenital ichthyosis; inherited in an autosomal dominant manner. The disease manifests itself immediately after the birth of a child in the form of a "colloidal fetus". After rejection of the film, the skin of the newborn gives the impression of being scalded. It is bright red in color, with extensive areas of detachment of the epidermis with the formation of erosions and blisters of various sizes, with a flaccid tire and a positive symptom of detachment of the bubble. The skin of the palms and soles is thickened, whitish in color, there is no ectropion. In severe cases, the process is accompanied by a hemorrhagic component (purpura) and is fatal. In milder cases, children survive. More often, with age, the number of blisters decreases sharply, and keratinization of the skin increases unevenly in different areas. At the 3-4th year of life, hyperkeratosis is clearly detected in the form of thick brown verrucous layers. The face is usually unaffected except for mild keratosis of the nasolabial folds; hair and nail growth is accelerated. On the skin of the trunk there may be needle-type hyperkeratosis, almost generalized, but uneven, more pronounced in the area of skin folds, where it takes the form of horny combs. The concentric arrangement of the scallops on the extensor surfaces of the joints is characteristic. Periodically, blisters appear on the skin, leaving erosions, the number of which is more pronounced in the first few years of life.

Histologically, epidermolytic proliferative hyperkeratosis, acanthosis, vacuolization of the cytoplasm of cells of the granular and prickly layers are revealed. The mitotic activity of the epidermis is enhanced. Histogenesis is based on a violation of the formation of tonofibrils, in connection with which intercellular communications are disrupted and epidermolysis is observed with the formation of cracks and lacunae.

Treatment. Retinoids (tigazon, neotigazon, etc.) at the rate of 0.5-1.0 mg / kg per day for 2-3 months or more (up to 1 year), depending on the clinical picture, with a gradual dose reduction. It is also possible to use repeated courses of vitamins A (400,000 IU / day), aevit, C, group B, biotin. Lipamide, methionine, ribosan, etc. are prescribed to normalize fat metabolism. In case of congenital ichthyosiform erythroderma in the neonatal period, corticosteroid hormones are prescribed (prednisolone at the rate of 0.75-3.5 mg / kg per day) in combination with antibiotic therapy, anabolic hormones, gemodez, which can significantly weaken the clinical picture of ichthyosis in the future. Hydroprocedures are shown: salt baths (100 g of sodium chloride or sea salt per bath) followed by rubbing 10% salt cream on lanolin and fish oil into the skin. starch (1 tablespoon of paste per bath), soda baths with bran, sulfide, carbonic, etc.; thalassotherapy, heliotherapy, silt and peat mud, UV rays in suberythemal doses, rePUVA therapy, immunotherapy (γ-globulin, etc.). I prescribe externally) - ointments with vitamin A (100,000 IU per 1 g of base), 0.1% tigazon cream, 2% salicylic ointment, 5% with urea, 1-20% ointment with malic, citric or glucuronic acid.

KERATODERMIA

Keratoderma (syn. keratosis palmar-plantar) is a group of keratinization diseases characterized by excessive horn formation mainly in the palms and soles.

By the nature of the clinical picture, xratoderma can be diffuse, with a continuous lesion of the entire surface of the palms and soles ( keratoderma Unna-Toast, Meleda, Papillon-Lefayvra and etc.). and localized, in which areas of excessive keratinization are located in foci (Buschke-Fischer-Brauer keratoderma, etc.)

Unna-Toast's keratoderma (congenital palmoplantar keratoma)- a common form of hereditary diffuse keratoderma, which is characterized by keratosis of the palms and soles without moving to other areas of the skin. The type of inheritance is autosomal dominant. The disease manifests itself in the first years of life in the form of a slight thickening of the skin of the palms and soles. Gradually, diffuse keratosis increases by the age of 4-5, rarely later. By this age, the clinical picture of the disease is completely formed. Horny layers on the palms and soles (sometimes only on the soles) are smooth, thick, yellow in color, with a sharply defined edge, which is surrounded by an erythematous corolla 1-3 mm wide. The process is accompanied by local hyperhidrosis. Histologically, orthohyperkeratosis, granulosis, acanthosis are revealed, in the dermis there is a small perivascular inflammatory infiltrate. Hair, teeth are not changed. Nails may be thickened (18% of cases) but not dystrophic. Osteoporosis and osteolysis of the phalanges, deforming arthrosis of the interphalangeal joints, complication of the process by a fungal infection are possible.

Keratoderma meleda (hereditary transgradient keratosis)- a form of hereditary diffuse keratoderma, characterized by the transition of keratosis from the palmar-plantar surfaces to the rear of the hands, feet, areas of the elbow, knee joints (transgradient keratosis). It is described for the first time among the blood relatives of the population of the island of Meleda. The mode of inheritance is usually autosomal recessive. The first manifestations of the disease occur in childhood in the form of persistent erythema with peeling of the skin of the palms and soles. In the future, keratinization of the skin intensifies, and by the age of 15-20, massive yellow-brown horny layers are visible on the palms and soles, lying in compact plates, erythema persists only in the form of a purple-purple rim several millimeters wide along the periphery of the focus. Histologically revealed hyperkeratosis, sometimes acanthosis, in the dermis - a small inflammatory lymphohistiocytic infiltrate. Local hyperhidrosis is characteristic, the surface of keratosis foci is usually moist, with black dots of the excretory ducts of the sweat glands. Horny layers pass to the back surface of the hands, feet, areas of the elbow and knee joints, painful deep cracks form on their surface (especially in the heel area). Characterized by a combination with atopic dermatitis, complications of the process of pyococcal infection, nail dystrophy with their sharp thickening or koilonychia. There may be changes in the electroencephalogram, mental retardation, syndactyly, folded tongue, gothic palate.

Papillon-Lefevre keratoderma (Papillon-Lefevre syndrome)- hereditary diffuse keratoderma, combined with periodontal disease and pyogenic infections of the skin and gums. The type of inheritance is autosomal recessive. Patients have a decrease in the function of the thyroid and pancreas, a violation of the functional activity of leukocytes, a decrease in the phagocytic activity of neutrophils and the sensitivity of T- and B-lymphocytes to mitogens. The clinical picture usually manifests itself at the age of 1 to 5 years (more often at the 2-3rd year of life) in the form of erythema of the palms and soles, covered with horny layers, the intensity of which gradually increases. Areas of keratosis often go beyond the palmar-plantar surfaces to the rear of the hands and feet, the area of the calcaneal (Achilles) tendon, knee and elbow joints. Characterized by localized hyperhidrosis. Histologically revealed hyperkeratosis, irregular parakeratosis, in the dermis - a small inflammatory infiltrate. In the cells of the horny and granular layers, lipid-like vacuoles, a violation of the structure of tonofibrils and keratohyalin granules are found. Nails are often dystrophic (dull, brittle), hair is not changed. At the age of 4-5 years, as a result of persistent gingivitis, progressive periodontal disease develops with the formation of purulent alveolar pockets, inflammation and degeneration of the alveolar processes with premature caries and tooth loss, an anomaly in their development. Possible calcification of the dura mater, arachnodactyly, acroosteolysis.

Buschke-Fischer-Brauer disseminated keratoderma (Buschke-Fischer disseminated keratosis)- the most common form of focal hereditary keratoderma. The type of inheritance is autosomal dominant. The first symptoms of the disease appear in puberty or somewhat later (from 15 to 30 years). Horny nodules appear on the skin of the palms, soles and flexion surface of the fingers - “pearls” ranging in size from 2 to 10 mm in diameter, which turn into dense horny yellowish-brown plugs with a crater-shaped edge. With the rejection of the central horny masses, a crater-like depression remains. Sweating is not disturbed. Histologically, hyperkeratosis with parakeratosis in the central part, slight acanthosis, and a slight perivascular inflammatory infiltrate in the dermis are revealed.

The diagnosis of keratoderma is based mainly on clinical findings. Differential diagnosis is carried out with various forms of keratoderma, psoriasis, dyshidrotic eczema.

Treatment: retinoids (tigazon, neotigazon, etc.) - 0.5 mg / kg per day for several weeks, aevit, angioprotectors (teonicol, trental, etc.); externally: keratolytic ointments (20% salicylic marinade. Arievich ointment). salt baths, phonophoresis with vitamin A. 20% dimexide, laser therapy. With Papillon-Lefevre keratoderma, treatment begins with antibiotic therapy and sanitation of the oral cavity.

EPIDERMOLIS BULLOSIS

Bullous epidermolysis (syn. Broca's hereditary pemphigus) is a group of hereditary bullous dermatoses characterized by the formation of blisters on the skin and mucous membranes that occur with minor trauma or spontaneously. There are several main types of epidermolysis bullosa based on the features of the mechanism of bladder formation and the clinical picture: simple, connective and dystrophic. Within these types, more than 10 different forms of the disease have been described.

Epidermolysis bullosa simple characterized by the formation of intraepidermal blisters as a result of disintegration and cytolysis of keratinocytes without signs of scarring, atrophy and the formation of milia. The type of inheritance is autosomal dominant.

clinical picture. The first signs of the disease usually appear in the 1st year of life, sometimes they can be already at birth. At the site of mild trauma, more often in the area of the hands, feet, back, elbow and knee joints, occipital region, blisters of various sizes (from 0.5 to 7 cm or more) with a dense cover and transparent contents appear on unchanged skin. Nikolsky's symptom is negative, there are no acantholytic cells in the contents of the bladder. After a few days, the blisters open, forming erosions, crusting and rapidly epithelializing, leaving no scarring of the skin or atrophy. There are usually more bubbles in the warm period of the year with severe hyperhidrosis. With age, lesions are localized mainly on the limbs, especially on the feet and hands, which is facilitated by a large traumatization of these skin areas. tight, ill-fitting shoes, as well as in areas of close fitting clothing. Bubbles appear throughout life, but in the post-pubertal period their number decreases. Mucous membranes, nails are not affected or their changes are minimal. The general condition of the patient does not change.

Prenatal diagnosis of this form of the disease is possible by the high content of α-fetoprotein in the blood serum of a pregnant woman in the II trimester. Epidermolysis bullous simple summer Weber-Cockane- abortive localized form of bullous simplex epidermolysis. It is characterized by the formation of blisters on the skin of the hands and feet only in the summer season with pronounced palmoplantar hyperhidrosis.

Epidermolysis bullous connective characterized by the formation of subepidermal blisters due to damage to the lamina lucida of the epidermal-dermal junction located between the plasma membrane of the basal keratinocytes and the basement membrane of the skin, and the development of atrophic skin changes in the lesions. Prenatal diagnosis is possible with a skin biopsy of an 18-week-old fetus based on the detection of these changes. The type of inheritance is autosomal recessive.

clinical picture. The process is characterized by the appearance of blisters and erosions already at the birth of a child or shortly after it. Within a few days, the process is generalized. The main localization of rashes is the skin of the chest, head, mucous membranes of the mouth, larynx, trachea. Although the skin of the hands and feet is not changed, the nail plates are dystrophic, anonychia and acroosteolysis develop. The erosive surfaces formed at the site of the blisters are poured slowly, leaving areas of skin atrophy. There are no scars or milia. Many children die in the first months of life from sepsis, anemia.

Epidermolysis bullous dystrophic characterized by the formation of blisters due to dermatolysis - the death of collagen fibrils in the papillary dermis below the lamina densa. Erosive and ulcerative surfaces are formed, healing with scars, the formation of milia, changes in nails, hair, teeth and other anomalies are also characteristic.

Epidermolysis bullous dystrophic recessive generalized (epidermolysis bullous dystrophic polydysplastic) differs in the formation of bubbles in the papillary layer of the dermis and the result of dermatolysis - lysis of collagen fibrils with phagocytosis of their macrophages and destruction below the lamina densa. The pathological process is associated with an increase in the level and activity of the collagenase enzyme, which destroys the main component of the supporting collagen fibrils - collagen VII (collagenolysis). Prenatal diagnosis of the disease is possible based on the results of a fetal skin biopsy at the 21st week of development and the detection of the previously described changes.

The first signs of the disease appear already at birth (60% of patients) or in the first weeks of life. Large blisters, often with hemorrhagic contents, occur spontaneously on any part of the skin and mucous membranes. Extensive long-term non-healing erosive-ulcerative surfaces that form when they are opened make it difficult to care for and feed newborns. The symptom of epidermal detachment is positive. Vegetations develop on erosive-ulcerative, often bleeding, painful areas. Their healing is slow, with the formation of disfiguring atrophic scars. Cicatricial changes in the esophagus, pharynx, oral mucosa can make it difficult to eat, obliterate the excretory ducts of the salivary glands, limit the mobility of the tongue and lead to the development of leukoplakia. Eye lesions in the form of erosive-ulcerative keratitis with subsequent scarring lead to loss of vision, cicatricial ectropion, obliteration of the ducts of the lacrimal glands. There are also acrocyanosis, scleroderma-like changes in the skin of the hands and feet with the formation of flexion contractures of the joints, acroosteolysis, and characteristic deformity of the hands as a “mitten” as a result of fusion and deformity of the fingers. Dystrophy of nails, hair, teeth is also characteristic. Possible violations of the endocrine (hypofunction of the thyroid gland, pituitary gland), nervous (epilepsy, mental retardation) systems. There is a high mortality in early childhood from sepsis, anemia, malnutrition, at an older age - from malignant neoplasms of the skin, esophagus, and oral cavity organs.

Epidermolysis bullous dystrophic dominant (epidermolysis bullous dystrophic hyperplastic) characterized by the formation of blisters in the dermis (dermatolysis) below the lamina densa due to the death of supporting collagen fibrils; prenatal diagnosis is possible (by analogy with dystrophic polydysplastic bullous epidermolysis). The type of inheritance is autosomal dominant. The first manifestations of the disease appear in early childhood or somewhat later (4-10 years). Bubbles occur after a minor injury, often in the limbs. They are tense, dense, with serous or hemorrhagic contents: when opened, they form erosive-ulcerative surfaces that heal slowly with the formation of mild or keloid-like cicatricial atrophy, initially pink, then white. In the area of the joints at the site of the blisters (extensive lesions are formed in the form of scar tissue with many epidermal cysts (milia). The symptom of epidermal detachment is positive. The nails involved in the process are thickened, dystrophic. Mucous membranes are rarely affected. Hair, teeth and general development are usually do not change, but often associated with ichthyosis, follicular keratosis, hypertrichosis Diagnosis of bullous epidermolysis is based on clinical and histological findings.Prenatal diagnosis of the disease is possible.Differential diagnosis in early childhood is carried out with epidermolytic ichthyosis, in which keratosis dominates; epidemic pemphigus of the newborn, which is characterized by an acute onset with fever, intoxication and inflammatory blisters as a result of necrotic processes in the epidermis caused by staphylococcus In older children, some forms of epidermolysis bullosa are differentiated from benign bullous pe mphigoid. which is distinguished by the linear deposition of IgA along the nasal membrane. Antibodies to lamina densa, type VII collagen, pemphigoid, etc. help to establish the nature of the defect and clarify the diagnosis.

Treatment is symptomatic. With a simple form of epidermolysis bullosa, it is important to protect the skin from trauma, exposure to high temperatures, tight clothes and shoes, and also from the addition of a secondary infection. Inside prescribe vitamins (A, group B, C), phytin; the blisters are opened and impregnated (without tearing off the tire) with aniline dyes, antibiotic ointments, epithelial ointments. In connective and dystrophic epidermolysis, in addition to these drugs, in severe cases, corticosteroid drugs (1-3 mg / kg per day) are prescribed in combination with antibiotics, iron preparations (tardiferon, etc.), red blood cell transfusions (for anemia), cardiac drugs Phenytoin is used to inhibit collagenase.

RECKLINGHAUSEN'S DISEASE

Recklinghausen's disease (neurofibromatosis type I) is a hereditary neurocutaneous phakomatosis. It is characterized by the development of multiple neurofibromas, neurinomas and other defects in the development of the ecto- and mesoderm.

Etiology and pathogenesis. It is caused by a mutant autosomal dominant gene located on chromosome 17, which has 100% penetrance, due to which the disease manifests itself in all children from the neonatal period to 5 years of age.

clinical picture.

The skin changes are characterized by multiple neurofibromas, café-au-lait spots, and a freckle type. Initially, rounded, sharply contoured (coloured coffee with milk) spots with a diameter of 0.5 to 10 cm or more usually appear due to the accumulation of melanocytes. Later, more often in the axillary fossae, small pigment spots are observed. Neurofibromas are benign tumors that develop from lemmocytes of peripheral nerves and are the most typical symptom of the disease. They usually appear later (after a few months or years, sometimes by puberty) and are soft lilac-pink, domed or sitting on a leg, rounded with a diameter of several millimeters to several centimeters. From light pressure, many tumors are drawn into the skin (symptom “Call buttons". The largest number (perhaps hundreds) is located on the trunk (especially on the chest, back, lower back). Soft fibrous nodules can be palpated along the peripheral nerves. A plexiform neuroma, a diffuse tumor that runs along the trigeminal nerve or other nerves and consisting of dense tortuous strands.In 5-10% of patients, papillomatous tumors form on the inner surface of the cheeks, tongue, lips.Abortive form of Recklinghausen's disease, characterized only by age spots, is called Leshke's syndrome.Kyphoscoliosis, cardiorespiratory pathology often develop; intelligence is often reduced , fall behind e physical development, endocrine disorders (gynecomastia; hyperparathyroidism, etc.), in 40% of patients, tumors of the central nervous system are detected.

Treatment. Surgical excision of individual tumors.

What it is? Hyperkeratosis is a thickening of the epithelium caused by an excess accumulation of keratin cells.

There are two mechanisms for the development of this state. The first is associated with increased keratin formation - proliferative hyperkeratosis. And at the heart of the formation of the second violation of desquamation of horny cells. At the same time, they are not rejected from the body, but accumulate, forming visible thickening of the skin - this is retention hyperkeratosis.

Affected by a pathological condition, the skin thickens, acquires a yellowish color, loses sensitivity and elasticity. Hyperkeratosis can be widespread (diffuse), affecting large areas of the body, or limited.

Perhaps one of the most striking examples of the second type of lesions are corns. Diffuse keratosis develops on the feet, back or limbs. With a genetic disease such as ichthyosis, pathological changes affect the entire body.

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Hyperkeratosis photo signs in humans

It should be noted that signs of hyperkeratosis can appear both in completely healthy people and in people with any diseases. In the first case, keratinization of the skin is caused by external - exogenous - factors:

constant rubbing of the skin with rough shoes or clothes;
wearing shoes that do not fit, high-heeled shoes with a narrow toe;
prolonged increased pressure on certain parts of the body (for example, feet);
regular aggressive exposure to the skin of chemicals;
insufficient hygiene;
aging;
harmful effects of UV rays.

Endogenous or internal causes of the development of hyperkeratosis include hereditary pathologies (ichthyosis, psoriasis, red hair lichen) and systemic diseases with a chronic course:

diabetes;
obesity;
hypofunction of the thyroid gland;
flat feet and other pathologies of the foot;
hypovitaminosis A and E;
atherosclerosis of the vessels of the lower extremities;
fungal infections of the skin and nails.

The most common keratinization of the foot or its individual parts: the heel, arch or "pads" - the place where the fingers are connected to the foot. In addition, there are other types of this pathological condition:

Subungual hyperkeratosis;
Hyperkeratosis of the squamous epithelium.

Hyperkeratosis of the skin - what is it?

When areas of keratinization develop on the skin, the clinical picture may vary in different conditions. Common features of all forms of skin hyperkeratosis are excessive dryness of the integument, coarsening and tuberosity.

The affected areas lose their sensitivity, but when pressed on them (corns), pain is possible due to irritation of the nerve endings by coarse tissues.

Follicular hyperkeratosis (photo)

On the elbows, knees, outer thighs and in the gluteal folds, the skin is naturally drier. The more keratinized scales are formed by the integument of the body, the more difficult it is for them to be rejected and easier to accumulate.

Dead cells in follicular hyperkeratosis cause blockage of hair follicles. Clogging into their mouths, epithelial scales cause inflammatory changes there, leading to the formation of dense tubercles.

If you run your hand over areas of the body affected by the follicular form of keratosis, a feeling of goosebumps will appear. Dense tubercles may be barely visible or stand out as bright red or brown spots.

Accidental injury or attempts at extrusion often lead to microbial infection of the follicles, their suppuration, and the development of complications -.

foot hyperkeratosis

Of all parts of the body, it is the foot that takes the most stress. It accounts for the entire weight of the body. It is easy to understand that with obesity, static and dynamic effects are amplified many times over. In addition, long walking, “standing” work, when a person has no time to rest, negatively affects.

On the other hand, people rarely think about their feet. Model shoes with high heels, the lack of proper correction of flat feet and other pathologies only exacerbate the situation.

Compensatory organism seeks to protect itself. In the epidermis of the feet, in response to adverse effects, horny cells begin to actively form, their number grows, the skin thickens, becomes insensitive, rough. If a woman constantly wears high-heeled shoes, the maximum load on the forefoot causes keratinization of this particular area.

Standing work is the key to hyperkeratosis on the heels. With flat feet, the middle part of the foot (arch) can coarsen. If a limited area is overwhelmed by excessive load or systematic friction or pressure, a callus will form.

Hyperkeratosis of the foot photo of cracks

In addition, hyperkeratosis always develops with a fungal infection. If the causative agent of mycosis has settled on the nail, subungual hyperkeratosis develops. This condition is characterized by an increase in the number of keratinocytes in the skin. As a result, clusters of these cells are formed, lifting the body of the nail, which looks like its thickening.

Frequent occurrence - keratinization of the skin of the feet in diabetics. It is caused by violations of tissue trophism. It is this category of people who should be more careful than others to monitor foot hygiene, carefully select shoes of a suitable size and high quality, and also use various correctors for the foot.

A fairly common complication of foot hyperkeratosis in advanced form is the formation of cracks. It is caused by a loss of skin elasticity combined with the accumulation of an impressive mass of keratinized cells.

Such cracks are characterized by impressive depth and are difficult to treat.

With the previously described forms of hyperkeratosis, pathological changes are visible to the naked eye. However, if a squamous epithelial lesion develops, no distinguishable signs are observed. This pathological condition affects women, or rather, the squamous epithelium of the cervix.

Normally, the processes of desquamation and the formation of new cells lining the mucous membranes of the genital organs are in balance. They are regulated by the hormones estrogen and progesterone.

With an imbalance of these substances, too intense cell death can occur. The gynecologist during the examination notices whitish spots on the cervix. However, the patient has no complaints. The final diagnosis of "hyperkeratosis of the squamous epithelium" (leukoplakia) is based on the results of a cytology smear.

In addition to hormonal disorders, a pathological condition can be provoked by such reasons:

human papillomavirus (HPV) infection;
chronic venereal infections and candidiasis;
consequences of cervical injuries (abortions, ruptures during childbirth, rough sex);
menopause;
drop in immunity;
intrauterine devices;
aggressive treatment of cervical erosion (diathermocoagulation).

Leukoplakia is considered a precancerous condition. It creates an optimal environment for the development of malignant neoplasms.

Allocate 3 forms of hyperkeratosis of squamous epithelium:

true;
parakeratosis;
dyskeratosis.

true form caused by hormonal imbalances, in particular, excessive production of progesterone.

Parakeratosis- a consequence of traumatic injuries of the cervix (during abortion, childbirth), as a result of which scars form on it. Connective scar tissue is inelastic, rough and easily prone to keratinization.

Dyskeratosis is the most dangerous form. If such a diagnosis is made, we are talking about a malignant degeneration of cells: they randomly divide, form growths and poorly exfoliate.

Hyperkeratosis of the squamous epithelium, unlike keratinization of the skin, poses a serious health hazard, requires careful monitoring and mandatory treatment.

Treatment of hyperkeratosis - drugs and methods

The tactics of treating hyperkeratosis is determined by their causes. If keratinization of the skin is caused by external factors, they should be eliminated or harmful effects minimized. Then the condition will improve.

Treatment of skin hyperkeratosis

Treatment of skin hyperkeratosis caused by systemic diseases or fungal infection should be aimed at eliminating the underlying pathology in the first place.

With hereditary diseases, it will not be possible to completely get rid of hyperkeratosis, you can only slightly smooth out its manifestations.

The general principles of skin keratinization therapy are reduced to the removal of dead areas and moisturizing the integument. With follicular hyperkeratosis, the effect should be gentle.

Scrubs and aggressive peels are prohibited, it is allowed to use products based on fruit or lactic acids. They contribute to the gentle removal of keratinization. Also useful is the local application of drugs with vitamins A and E, as well as their course intake.

With hyperkeratosis of the feet, it is important to eliminate all traumatic effects. You can remove coarsened tissues with special files, blades, grinders both at home and in a beauty salon.

Useful creams with urea (Foretal-Plus), with fungal infections - antimycotics (Loceril, Lamisil).

Treatment of hyperkeratosis of squamous epithelium

Therapy for leukoplakia should be comprehensive. In chronic genital infections, courses of antibiotics and antiviral drugs are indicated. In menopause or with endocrine disorders, hormonal levels should be adjusted.

But whatever the cause of hyperkeratosis of the squamous epithelium on the cervix, the removal of dead areas is necessary. In the arsenal of gynecologists are modern painless remedies:

cryodestruction (cauterization with liquid nitrogen);
cauterization with a laser;
radio wave therapy.

Solkovagin is often used to cauterize keratinized areas of the squamous epithelium. This agent dissolves foci of hyperkeratosis when applied topically.

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1) psoriasiform type - uniform elongation of the epidermal processes while maintaining the dermo-epidermal configuration;
2) diffuse type - uniform hyperplasia of the entire epidermis;
3) papillary type - focal growths of the epidermis, protruding above the surface of the skin;
4) pseudocarcinomatous type - massive uneven hyperplasia with penetration of the epidermis into the hypodermis, resembling squamous cell carcinoma.

Acanthosis is observed in many skin diseases, in particular with malformations (warty nevus), erythrokeratoderma, palmoplantar keratoderma. Often, acanthosis develops a second time in various chronic inflammatory processes, especially those accompanied by itching (, chronic, dermatitis, etc.).

- thinning of the epidermis due to a decrease in the number and vertical diameter of spiky cells.

Atrophy- thinning of the epidermis due to a decrease in cell size. The epidermal processes in these cases are smoothed down to the formation of a rectilinear form of the epidermal-dermal junction.

Necrosis- cell death characterized by karyopyknosis (wrinkling of nuclei), karyorrhexis (fragmentation of nuclei) or karyolysis (complete disappearance of nuclei).

Necrosis of epidermocytes may be focal or total. Trauma, impaired blood supply can lead to severe and complete necrosis of the epidermis. Epidermal necrosis, expressed to varying degrees, is noted in erythema multiforme exudative, Stevens-Johnson syndrome, and acute guttate parapsoriasis. In these cases, it is usually combined with a pronounced inflammatory reaction. In Lyell's syndrome, however, severe epidermal necrosis occurs without significant signs of inflammation.

Focal necrosis basal keratinocytes is an important symptom of lupus erythematosus, acute lichen planus, fixed, melasma, lichenoid toxic reactions, vascular poikiloderma. In acute solar dermatitis, scattered cells with pycnotic nuclei appear in the middle sections of the epidermis.

Anaplasia(dysplasia,) - varying degrees of atypia of epidermal cells in malignant and premalignant epithelial tumors of the skin. The nuclei of anaplastic cells are usually large, hyperchromic, and irregularly shaped; they show atypical mitotic figures.

Hyperkeratosis (orthokeratosis)- thickening of the stratum corneum of the epidermis. Hyperkeratosis is represented by an excess of normal-looking horny cells. There are proliferative and retention (follicular) hyperkeratosis. Proliferating hyperkeratosis is characterized by hyperplasia of the horny, granular and prickly layers of the epidermis; it is observed with lichen planus, keratoses. Proliferating hyperkeratosis in ichthyosis is characterized by a delay in the rejection of horny cells, which leads to a thickening of the stratum corneum; the granular and often spiny layer becomes thinner at the same time.

Retention (follicular) hyperkeratosis- noted in the mouths of hair follicles, it sometimes leads to rupture of the walls of the follicles. This symptom is most pronounced with the follicular form of lichen planus, lichen pilaris, sclerotic and atrophic lichen, as well as other dermatoses associated with the pathology of the hair follicles.

Parakeratosis- defective keratinization of the epidermis, in which parakeratotic cells containing pycnotic rod-shaped nuclei are formed in the stratum corneum. The causes of parakeratosis are inflammatory changes in the prickly and granular layers of the epidermis, as well as accelerated proliferation of keratinocytes.

Parakeratosis is observed in the following pathological processes:
1) inflammatory dermatoses (eczema, dermatitis, etc.);
2) skin regeneration after various injuries;
3) dermatosis, accompanied by increased proliferation of keratinocytes ();
4) some precancerous conditions and intraepidermal cancers (actinic keratosis, Bowen's disease, etc.).

Dyskeratosis- Autonomous, defective and premature keratinization of individual keratinocytes. There are two types of dyskeratosis: acantholytic (benign) and neoplastic. Acantholytic dyskeratosis is observed in Darier's disease, warty dyskeratoma, actinic keratosis, sometimes in transient acantholytic dermatosis, and familial benign pemphigus Hailey-Hailey. Keratinocytes in these cases turn into so-called "round bodies" (homogeneous, basophilic, pycnotic nuclei surrounded by voids in the cytoplasm, as well as "grains" (round formations with sharply eosinophilic cytoplasm and a small pycnotic nucleus). "Grains" are usually located in the horny layer of the epidermis.

Neoplastic dyskeratosis is also a consequence of individual keratinization of keratinocytes. It appears as homogeneous eosinophilic masses, sometimes containing remnants of the nucleus. This type of dyskeratosis is seen in Bowen's disease; actinic keratosis; squamous cell carcinoma, especially its pseudoglandular and adenoid variants. It can also occur in keratoacanthoma and tricholemma of the scalp.

Hypergranulosis- an increase in the number of cells of the granular layer, usually accompanying orthokeratosis. Hypogranulosis - a decrease in the number of granular keratinocytes; agranulosis - their complete absence.

What it is?

Hyperkeratosis is a condition of the surface layer of the skin, expressed in an increase in the number of keratin cells of the stratum corneum of the epidermis without changing their structure, leading to a thickening of the skin of the affected area. With hyperkeratosis, the skin loses its elasticity, becomes less extensible (rigid), and its sensitivity to external stimuli decreases.

The concept of hyperkeratosis is associated not only with skin changes. With the development of a pathological process in the epithelial cells lining the cervix, they also speak of the presence of hyperkeratosis.

Almost any person with age or under the influence of adverse factors may experience increased physiological keratinization of the skin of the feet and palms. People suffering from internal metabolic diseases, and especially, are at a higher risk of developing hyperkeratosis.

skin hyperkeratosis photo

Hyperkeratosis is widespread with the most common skin disease - and nail fungus, which is present in every fifth person on Earth.

Hyperkeratosis of the epithelium of the genital organs occurs, according to different authors, from 5 to 12.5% of all diseases of the cervix.

Kinds

photo of a diffuse form of hyperkeratosis

According to the mechanism of formation, hyperkeratosis occurs:

Excess formation of keratin - proliferative hyperkeratosis;
Delayed rejection of horny cells - retention hyperkeratosis.

By prevalence:

Limited - calluses, keratomas, warts;
Widespread or diffuse - large surfaces are affected, such as the skin of the feet, back, limbs, or the entire body, which is observed with ichthyosis.

Follicular;
Hyperkeratosis of the feet;
subungual;
seborrheic;
Hyperkeratosis of the cervix.

Causes of hyperkeratosis

Endogenous factors, that is, originating within the body, contribute to changes in the work of the cells of the surface layer of the skin. This process can only be influenced by the treatment of the causative disease, which will slow down the rate of formation of keratinizing cells and improve their exfoliation.

internal reasons may include the following diseases and conditions:

Hereditary, genetic predisposition;
Diabetes;
Atherosclerosis of the vessels of the legs;
Flat feet, (“bumps” of the big toes), diseases of the ligamentous apparatus and deformities of the bones of the legs;
Fungal infections of the skin and nails;
Ichthyosis, keratoderma, psoriasis, Devergie's disease (red hair lichen);
Insufficient production of thyroid hormones ();
Hypovitaminosis A.

Exogenous factors, that is, those outside the body and not dependent on it, are the most common. The most common cause is excessive pressure on the skin cells, which stimulates the growth of keratin cells and leads to thickening of the skin.

When this effect is eliminated, the normal process of cell formation and desquamation is restored.

External causes include:

Tight, uncomfortable shoes;
High heel;
Excessive pressure on the foot due to excess weight;
Friction of clothes;
Excessive passion for tanning, leading to accelerated aging of cells;
Decrease in the natural pH level of the skin when using aggressive detergents;
Smoking - acts on the skin from the inside, but is initiated by a person from the outside.

The combination of exogenous and endogenous causes provokes the development of hyperkeratosis especially actively. For example, wearing uncomfortable shoes for patients with diabetes and obesity.

Causes of cervical hyperkeratosis:

Menstrual disorders: oligomenorrhea, insufficiency of the 2nd phase of the cycle, dysfunction of the pituitary-ovarian system;
Transferred earlier, inflammatory diseases of the genital organs;
Recurrent pseudo-erosion, as well as their excessive treatment, diathermocoagulation, especially without indications.

The occurrence of signs in this type of hyperkeratosis is due to the clogging of the mouths of the hair follicles with keratinized scales, which leads to the formation of characteristic dry cones-thorns, which, when probed, create a “grater syndrome” due to the uneven surface and sharpness of the spines.

These formations are small and hardly visible to the eye. The usual location for follicular hyperkeratosis is the skin of the back of the shoulders, infragluteal folds, knees, outer thighs, elbows, that is, areas of the skin that are initially prone to dryness.

Usually, the lesions are painless and do not bring significant discomfort. With a widespread lesion, follicular hyperkeratosis of the skin can bring aesthetic discomfort.

Injury to horn formations is dangerous by infection and the development of inflammation of the mouth of the follicle. In this case, there is the appearance of dotted redness, pustules and soreness of the affected area.

Foot hyperkeratosis or plantar hyperkeratosis

Signs of hyperkeratosis of the feet:

dry corn- most often located in places that are reference points of bones: heels, space under the fingers in the area of the metatarsophalangeal joints (where the foot connects to the fingers).

The skin in these places experiences increased pressure, being sandwiched between the supporting surface and the bone. As a result, a formation with a clear boundary is formed, consisting of compressed keratinized scales.

Calluses are characterized by uniform thickness and yellow color, which can be accompanied by painful sensations, especially with increased pressure on the affected area and the formation of cracks.

callus- is a dry callus, in the center of which a translucent dense rod is formed, consisting of horny masses.

The edges of the callus are smooth, the boundaries are clear. Characterized by a sharp soreness with vertical pressure, which is due to the impact of the horny rod on the nerve endings inside the skin.

The areas of the sole in the area of the metatarsophalangeal joints, the so-called "ball of the foot", the lateral areas of 1-5 fingers, the skin of the toes on the back of the foot above the joints between the phalanges, especially with the "hammer-shaped" deformation of these fingers, are prone to the formation of calluses.

soft corn it is localized between the fingers, where there is increased humidity, therefore, areas of ulceration (maceration) appear on it, causing constant severe pain, burning.

Often complicated by the addition of infection and the development of inflammation.

cracks are a complication of widespread hyperkeratosis of the foot, usually develop around the heel. Cracks can bleed, become wet, become infected, and cause severe pain and discomfort.

squamous form mycosis of the feet, observed in diabetes mellitus. With this form, flat dry plaques and thickened spots (papules) appear on the skin of the arch of the foot, having a blue-red color and covered with gray scales.

Along the edge of the plaques, a kind of “border” of exfoliated cells can be detected. These rashes can combine and occupy a large area, and sometimes the entire sole, back and sides of the foot. Together with such manifestations, patients may have dry yellow calluses with cracks.

For subungual hyperkeratosis The most common cause is fungal infection of the nails. It is also observed in injuries and dystrophy of the nail plate. A sign of the pathological process will be a gradual thickening of the nail, starting from its edge.

In response to the multiplication of the fungal flora, keratinocytes begin to divide intensively and form a mass of gray-yellow horny epithelium, which accumulates under the nail plate, lifting the latter and creating a visible thickening of the nail.

Subungual hyperkeratosis can occur not only on the feet, but also on the hands.

Seborrheic hyperkeratosis

It is manifested by the appearance of spots, plaques and nodules, ranging in size from 2-3 mm to several centimeters, which grow very slowly. The foci have clear, well-defined boundaries, the surface is uneven and resembles warts.

The color of the formations is brown, from light to dark, on top they are covered with horny plugs and dry keratin.

Hyperkeratosis of the squamous epithelium of the cervix is detected only during a gynecological examination. It usually does not cause any symptoms and discomfort, a small number of patients may have abundant clear discharge.

To confirm the diagnosis, a scraping of epithelial cells is taken, which is sent for cytological examination.

Treatment of skin hyperkeratosis, drugs

Common in the treatment of hyperkeratosis of various forms will be the impact on the cause of the appearance. The patient needs consultations of a dermatologist, endocrinologist, therapist, in some cases - an orthopedist and a surgeon.

1. Treatment of follicular hyperkeratosis implies:

The use of vitamins A and E externally in the form of ointments and inside, in the form of oil solutions;
Peeling with fruit and lactic acids, which exfoliate dead cells well;
Moisturizing lotions and creams.

The use of harsh pumice stone cleansers and scrubs with solid particles can lead to trauma and festering of the follicles, therefore categorically contraindicated.

2. Treatment of foot hyperkeratosis:

Elimination of excessive pressure on the foot;
Selection of comfortable shoes;
Regular removal of rough skin on your own with the help of scrubs, files, grinding with a machine, special blades, or in a pedicure room;
Patients with diabetes for the treatment of feet are contraindicated in the use of sharp objects: scissors, blades, due to reduced skin sensitivity and a high risk of injury. You can use files, scrubs, soft softeners, pumice and scrapers on your own.
The best option would be the opportunity to visit a podiatrist (who deals exclusively with the treatment of feet) at the “Diabetic Foot” office for hygienic treatment of the feet;
External treatment with urea preparations: a concentration of 2-10% gives a good moisturizing effect, a concentration of 40% has a pronounced keratolytic and antifungal effect (used in the complex treatment of mycoses). Cream "Foretal-plus" well relieves rough skin on the heels due to the combination of urea at a concentration of 25% with phospholipids, which makes it effective for softening horny layers and moisturizing the skin.
- the use of antimycotic drugs inside: introconazole, terbinafine; removal of the affected part of the nail (set "Mycospor"); local application of therapeutic varnishes Lotseril, Batrafen, ointments and creams Lamisil, Exoderil, etc.

3. Treatment of seborrheic hyperkeratosis consists in carrying out various types of coagulations:

laser;
Cryo;
Chemical;
Electrocoagulation.

4. Treatment of cervical hyperkeratosis:

The elimination of the inflammatory process, depending on the type of pathogen, is used: antibiotics, antiviral, antifungal, antichlamydia and other drugs;
Laser coagulation is a painless and most effective method, which is recommended to be preferred;
Cryotherapy is also a painless and very effective method, but it has a drawback - a rather frequent return of the pathological process in patients with a disturbed cycle;
The effectiveness of 74% has the use of Solkovagin, which has a local chemical effect. Treatment painlessly and completely destroys the focus of hyperkeratosis;
Surgical treatment is carried out with pronounced changes in the cervix.

Widely used diathermocoagulation is currently considered as a possible, but undesirable method, which has many negative consequences.

It is necessary to refrain from using agents that stimulate cell growth: rosehip oil, ointments with aloe, sea buckthorn oil. The use of these drugs can provoke increased cell division and growth and lead to cervical dysplasia.

ICHTHYOSIS

X-linked ichthyosis is also characterized by deep stromal cataract, cryptorchidism, small testicles, penis, infertility, and mental retardation are possible.

Treatment. Retinoids (tigazon, neotigazon, etc.) at the rate of 0.5-1.0 mg / kg per day for 2-3 months or more (up to 1 year), depending on the clinical picture, with a gradual dose reduction. It is also possible to use repeated courses of vitamins A (400,000 IU / day), aevit, C, group B, biotin. Lipamide, methionine, ribosan, etc. are prescribed to normalize fat metabolism. In case of congenital ichthyosiform erythroderma in the neonatal period, corticosteroid hormones are prescribed (prednisolone at the rate of 0.75-3.5 mg / kg per day) in combination with antibiotic therapy, anabolic hormones, gemodez, which can significantly weaken the clinical picture of ichthyosis in the future. Hydroprocedures are shown: salt baths (100 g of sodium chloride or sea salt per bath) followed by rubbing 10% salt cream on lanolin and fish oil into the skin. starch (1 tablespoon of paste per bath), soda baths with bran, sulfide, carbonic, etc.; thalassotherapy, heliotherapy, silt and peat mud, UV rays in suberythemic doses, rePUVA therapy, immunotherapy (α-globulin, etc.). I prescribe externally) - ointments with vitamin A (100,000 IU per 1 g of base), 0.1% tigazon cream, 2% salicylic ointment, 5% with urea, 1-20% ointment with malic, citric or glucuronic acid.