Ichthyosis, forms of ichthyosis


  • forms ichthyosis
  • Diagnosis ichthyosis
  • The treatment of ichthyosis

  • Ichthyosis and ichthyosiform dermatoses (ichthyosiform erythroderma)
    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).

    forms ichthyosis

    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.

    Ichthyosis, forms of ichthyosisSimple (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)
    mental retardation.

    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 (stratification of the epidermis with the formation of blisters: 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.

    Diagnosis ichthyosis

    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

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