Vulgar ichthyosis: symptoms, treatment, diagnosis, forms

August 16, 2017 23:00 | Hereditary

With regard to skin diseases, vulgar ichthyosis is a serious lesion of the epidermis of the skin with pronounced manifestations and a variety of concomitant symptoms.Modern methods of treatment using multicomponent drugs can significantly reduce the unpleasant manifestations of the disease, but the treatment of vulgar ichthyosis is usually long.This article will tell you in detail about the symptoms and signs of vulgar ichthyosis in an adult and a child, their treatment and inheritance.

Features of the disease

Presenting a skin disease in which there is a violation of the keratinization process, vulgar ichthyosis occurs in both men and women in the same way: the epidermis loses uniformity in the keratinization of young cells, some of its areas thicken,And curing of the skin. In this case, this skin change can occur both in limited areas( to be limited) and widespread and unlimited in area( diffuse).

The process of keratinization of the cells of the epithelial layer with vulgar ichthyosis is somewha

t different from the usual: when dividing the germ cells, they are transferred to the upper layer of the skin.At the same time, under normal conditions, the lower layers are gradually displaced by the old ones, which causes an inconspicuous peeling of the skin.

This is a change in the life cycle of skin cells, as there is a movement of epithelial cells with accumulated substances in them in the surface layers of the epidermis.This leads to a gradual thickening of the skin, its coarsening.This slows the rejection of dead cells, which also leads to an increase in the thickness of the upper layer of the epidermis.

Common vulgar ichthyosis in children( photo)

Vulgar-common-ichthyosis-y-children

Today, many different forms of this skin condition have been identified, in which there is a disruption in the thickness of the upper layer of the skin, and their diverse morphological pattern makes it difficult to develop a single classificationDisease.The general term "ichthyosis" implies the presence of a significant number of skin diseases, in which there is a gradual thickening, a change in the processes of keratinization of the epidermis, as well as a violation of the rate of rejection of necrotic cells.

The lack of a unified view of the pathological conditions of the skin, a large number of diseases with similar manifestations, made it difficult to introduce a unified classification of ichthyosis.

According to the type of condition

In accordance with the teachings of such outstanding experts in the field of skin diseases, as VT Kuklin and IIPototsky, ichthyoses can be subdivided( very conditionally) into the following varieties:

  1. pathological conditions of , the cause of which should be considered gene mutations.They are usually referred to as genodermatosis, or congenital ichthyosis;
  2. conditions similar to ichthyosis are those forms of skin diseases with similar manifestations that are acquired during life and are called ichthyosiform.

These same specialists separately distinguish a subgroup of hereditary syndromes in which thickening of the skin( hyperkeratosis) is one of the most obvious symptoms:

  • Refsum syndrome;
  • Jung-Vogel syndrome;
  • Sjogren-Larson syndrome;
  • Popov's syndrome.

Symptom

Today in the course is also a classification of a variety of disease that was acquired during life.It is based on the symptoms and manifestations of hyperkeratosis and looks like this:

  1. is a symptomatic acquired ichthyosis , which is usually provoked by a particular pathology or may become a manifestation of a disease that may include abnormalities in the functioning of the thyroid, adrenal, avitaminosis, autoimmune, inflammatory diseasesAnd infectious, metabolic disorders, allergy to food or medicines;
  2. discoid acquired ichthyosis;
  3. senile , or senile ichthyosis.

Outward manifestations

Vulgarnyj-ihtioz-vid In 1990, Suvorova KNA classification based on the external manifestations of the disease was proposed and is based on the clinical manifestations of ichthyosis.According to this classification, the following forms of the disease should be divided:

  • ordinary , or simple ichthyosis.For this form of the disease is characterized by the defeat of the entire skin of the body, in which there are many small scales;
  • shiny variety - almost all skin integuments are affected, except for large folds of the skin.The grayish color of the scales is arranged in a mosaic pattern;
  • serpentine - skin damage occurs only on the extensor surfaces and the lateral surface of the body, and the scales have a pronounced brownish color.

Another type of classification is based on the degree of severity of the underlying disease, and it is the shape of the ichthyosis that is determined by it:

  • heavy - resulting in the death of a newborn;
  • late - manifestation of the first symptoms is observed during the second month from the moment of birth;
  • is a moderate - this disease is compatible with life.

The classification methods listed above allow for a better understanding of the nature of the disease and provide the possibility of classifying it as a specific type, thereby facilitating the choice of the treatment system.

Causes of

Vulgar ichthyosis refers to a simple form of ichthyosis, its development is directly related to the presence of gene changes in the body and it is inherited by an autosomal dominant type of inheritance.At the same time, mutated genes are inherited, they can control enzyme systems and the process of skin keratinization.Completely this mechanism is not deciphered.

With gene mutations, a change in the process of skin moisturizing, an increase in the amount of defective keratin produced, which is considered a protein of nails, hair and skin, as well as excessive cornification of the epithelium.In the case of metabolic disturbances in the body, which is especially typical for fat and protein disorders, the appearance of violations of the barrier functions of the skin occurs, which leads to a gradual accumulation of cholesterol inside the cell tissues.This gives the so-called cementing effect, thickening the skin, disrupting the process of its breathing, which significantly reduces the human immunity and negatively affects the work of the immune, endocrine systems.

Symptoms

External symptoms of vulgar ichthyosis include symptoms such as uneven skin peeling, thickening of the upper layer, changing tactile sensations in altered areas of the skin.The mechanism of activation of the disease is as follows:

  1. excess keratin production, which is accompanied by a change in the structure of the skin;
  2. increase in the rate of movement of keratinocytes into the upper layer of the epidermis;
  3. slowing the rejection of dead cells by strengthening the bonds between them when accumulating decay products in cell tissues;
  4. acquisition of cells of epithelium of a dystrophic nature, the appearance of vesicles( vacuoles) in the upper layer of the skin, a change in the thickness of the epidermis.

Further, the formation of filargin occurs, which leads to excessive excretion of water and, as a consequence, peeling of the skin and its dryness.

  • With the development of vulgar ichthyosis, hair falls out, and the peeling is mucoid, pearly or finely lamellar.Friability and thinning of the nail plates are also often observed, and skin lesions occur throughout the body, except for the side surfaces, gluteal and cervical regions.
  • The color of the scales can vary: from gray to whitish and black.The front surface of the shin is covered with scales in the form of fish scales and has a shiny surface.

Diagnosis of vulgar ichthyosis

Vulgar-ichthyosis features Ordinary ichthyosis can be diagnosed already with an external examination of the patient's skin with complaints of increased dryness of the skin, thickening of the skin, hair loss.The most characteristic manifestation of vulgar ichthyosis is the destruction of the hair follicle mouths by the masses of the horny epithelium, thus forming bumps of corpuscle or reddish-gray color, covered with scales of skin and occasionally surrounded by a reddish aureole.

However, the exact diagnosis is difficult due to the large blurred external inspection.Therefore, for more accurate diagnosis, carry out a number of additional studies, which, however, are very expensive.Their low availability also makes it difficult to diagnose the disease and clarify its variety.Therefore, in the presence of characteristic manifestations, great attention is paid to the following diagnostic actions:

  • consideration of the pedigree relatives of for the presence of such skin diseases, since the presence in the genus of patients with ichthyosis in any form is an indicator for the detection of this disease in subsequent generations;
  • the age of the patient , at which the symptoms of the disease began to appear, as well as the ratio of seasonality - usually vulgar ichthyosis with the onset of the cold season has more vivid manifestations than in warm weather;
  • The presence of such diseases as , as lesions of the gastrointestinal tract, endocrine and oncological diseases, deviations in the functioning of the biliary tract, intestine, pancreas, because they can be the cause of the development of ichthyosis of any shape, and be concomitant diseases.

With the help of laboratory research, it becomes possible to identify comorbid pathologies, to determine the immune status of the diseased.

Treatment

Reducing the occurrence of ichthyosis and improving the condition of the skin allows you to correct the patient's condition, but you should know that there are no special treatments for a complete cure for this disease.Therapy of the disease consists in the use of vitamin A, which improves both the general condition of the skin, and ensures the normalization of the process of keratinization of the epidermis.

Therapeutic method

In parallel with the medicinal treatment methods, the following recommendations should be observed:

  • regular care for the affected skin with moisturizers and nutrients that must also contain vitamin A or its derivatives;
  • reception of warm baths( water temperature not more than 38 ° С).Do not use soap, take a shower of cool temperature.It is recommended to use gels that contain natural oils and extracts of medicinal plants, desmised naphthalene.An excellent addition to the bath will be saline or starch, broths of chamomile and string;
  • after taking warm baths should use cream with high content of vitamin A;
  • rinsing affected areas with herbal infusions can help reduce the symptoms of the disease.Suitable camomile, motherwort, celandine, plantain.

By medicamentous method

The course of treatment with vitamin A is to take a daily dose of 4-5.5 thousand units( based on 1 kg of body weight).The duration of vitamin A is at least two months, after which a break for three weeks, then, if necessary, the course is repeated.

Complex therapy is also carried out with the use of preparations containing zinc: Tsinketral, Zinc, Tsinkit - these drugs have proven themselves in the treatment of ichthyosis and skin condition improvement.They are taken in combination with vitamin C and E - so zinc from medicines is absorbed better, and the effectiveness of therapy increases.

Prevention of the disease

Vulgar-ichthyosis-treatment To prevent the activation of pathological processes on the skin, one should carefully consider the appearance of changes in its condition, and also undergo a dermatologist's examination in time.

Complications

In the absence of treatment or improper selection of a method of therapy, the deterioration of the manifestations of the disease is likely, which significantly worsens the overall quality of life of the patient. There may be skin edema, weakening of its functions, and in infancy, with the active development of complications, a fatal outcome is likely.

Forecast

With timely treatment and proper selection of a method of therapy, it is likely to improve the skin condition, eliminate its pronounced peeling.

Lifetime in the presence of serious complications with improper or inadequate treatment may decrease: the smaller the age of the patient, the greater the impact this disease has on the duration of his life.Much also depends on the form of the disease, the level of resistance of the body and the work of the immune system.

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