Lamellar( plate) ichthyosis: treatment, diagnosis, photos of patients, ICD-10 code

August 18, 2017 23:00 | Hereditary

Skin lesions are accompanied by pronounced manifestations, which significantly reduce the quality of human life.And among skin diseases it is necessary to distinguish lamellar ichthyosis, which occurs in both women and men, can affect the upper layer of the epidermis at any age and does not have methods of definitive cure.This disease is inherited, can be detected at a very early age for a variety of external signs, which are confirmed by additional laboratory research.

Features disease

the classification of different forms of lamellar keratosis( lamellar ichthyosis of the skin), his view is worth a special place because of the presence in its current specific features.As well as other types of ichthyosis, the disease under consideration manifests itself in the form of skin lesions, which appear in the process of keratinization of its upper layer. epidermis with lamellar ichthyosis with active accumulation of fat in the blood and amino acid loses its elasticity and uniformity, which leads to a gradua

l change in its structure: there are places denominated seals, forming keratomas in the form of small-sized entities with a scaly surface.

Manifestations of skin disease lamellar ichthyosis are similar in both men and women.Dry skin, loss of elasticity and elasticity of the upper layer of the skin, dehydration and subsequent death of cells with the formation of solid scales, - the symptoms of this disease make it possible to make a preliminary diagnosis.At the same time, due to his hereditary possible transmission, lamellar ichthyosis is equally manifested in the female and male half of the population.

Lamellar ichthyosis( photo)

Lamellyarnyj-ihtioz-foto

Classification lamellar ichthyosis

separation classification of skin disease is dependent on the severity:

  • severe , at which the symptoms are obvious, evident atExternal examination of the patient's body.Also called a gastricoid form.It can be characterized by the appearance of a premature baby, with such children dying during the first days of life;
  • medium-heavy form - children tend to survive;
  • late form of the disease - manifestations of the disease are detected with some delay in time.

Causes

Today lamellar ichthyosis causes are not fully identified. However, the most real reasons are violations in the process of keratinization of the epidermis.This can occur because of gene mutations that can be inherited. In this case, if the direct descendants of parents who suffered from lamellar ichthyosis, this disease was not detected, then their children are no longer potential carriers of the mutated gene.

is also a likely cause of this skin disease Medco include the following factors:

  • disturbances in protein metabolism, in which the accumulation of amino acids observed;
  • abnormalities in fat metabolism - accumulates excess cholesterol;
  • changes in the process of body thermoregulation, resulting in an increase in the activity of various enzymes and the rate of oxidative reactions involved in diffuse gas exchange;
  • decreased activity of hormones - the thyroid gland, endocrine glands lose the ability to work actively, with the development of hypogammaglobulinemia, which is expressed as the emergence of a deficiency of the humoral link;
  • there is a deficiency in the body of vitamin A and its derivatives, which are responsible for the general condition of the skin.

To conduct an accurate diagnosis and appropriate treatment, which will remove the symptoms of the disease, additional studies are underway, as there are many varieties of ichthyosis today.

Symptoms

This skin disease affects the upper layer of the epidermis, the keratinization of young cells is disrupted, which is manifested in skin changes:

  1. on the surface of the skin, especially in the neck and face, as well as on the chest, areas with small dry scales form between the scapula, which eventually change the bodies from the color of healthy skin to grayish and dark gray;
  2. all the body of the child with this disease is covered with a thin film, which has a yellowish-brown hue, which can transform and take the form of the smallest scales;
  3. skin acquires an unhealthy appearance, affected areas with a mild form of the disease localized.The exception of the lesion is deep folds of the skin, the lateral surface of the body.However, with the course of the disease manifests itself even in those areas that are usually not subject to change;
  4. flakes with the activation of the disease can take the form of scutes that give the skin the appearance of a snake;
  5. lesions of nail plates with lamellar ichthyosis are rare, but sometimes it can be observed the appearance on the nails of strips of red or white flowers, the edge of the nail is thinner and begins to crumble.

For the removal of these symptoms requires timely diagnosis, which allows you to make the right scheme for treatment and removal of unpleasant manifestations.

Diagnostics

Lamellar ichthyosis Since the external manifestations of lamellar ichthyosis are quite noticeable, even a general examination of the affected parts of the body allows you to get a preliminary idea of ​​the existing disease.However, the specified diagnosis can be made only with additional laboratory tests.

Active sweating in this disease is typical in those areas that are affected: denser and red skin has a disturbed process of thermoregulation, which leads to sweating.The skin is stretched, actively scaly, which can become a sign of lamellar ichthyosis during diagnosis.

Next, you will learn how to treat lamellar ichthyosis of the skin in an adult and a child in a home and stationary setting.

Treatment of

It should be noted that there is no complete cure for this disease;However, modern drugs and an integrated approach can significantly improve the overall condition of the patient and restore skin health.After diagnostic studies, the entire course of treatment is performed only under the supervision of a dermatologist.

Therapeutic way

The severity of the disease determines the approach to treatment;In severe and neglected forms of the disease, treatment is recommended in a hospital.Usually, the course of treatment includes the intake of vitamin A and its derivatives, which have a positive effect on the skin condition.

Vitamin A is usually prescribed for all forms of ichthyosis, and its amount depends on the severity of the course of the disease.Its duration is from 3 to 5 weeks.Then follows a one-week break, after which the reception can be resumed depending on the skin condition.

Even after effective treatment, the redness on the skin can remain for a long time, in some cases - for life.

By medicamentous method

For external use with pronounced lamellar ichthyosis, agents that eliminate excessive dryness of the skin and peeling can be used.It can be Vaseline, a cream for external use Demato.They are applied to the cleared areas of the affected skin and left until soaking.Increasing the elasticity of the skin, reducing the production of active substances in the epidermis and lipid saturation of the skin are the main manifestations of the augmentation of the listed products.

Corrective hormonal background measures to improve the condition of the affected skin.Since often in this disease there is a change in the functioning of the thyroid gland, an endocrinologist can be prescribed hormone therapy to normalize the thyroid gland.These drugs include:

  • Thyreocom;
  • thyrotome;
  • Thyroidine.

Prevention of the disease

Lamellyarnyj-ihtioz-foto-2 As prevention, counseling may be recommended for those couples who have a history of lamellar ichthyosis and are planning a pregnancy. This will take me to lower the risk.

Regular hygiene procedures with the use of funds with a high content of vitamin A will help maintain the normal condition of the skin, its elasticity.This is especially important in the initial stage of the disease.

Complications

The complications of lamellar ichthyosis include the transition of the initial form to a more neglected one, when the symptoms are expressed as much as possible and cause discomfort to the patient.Increased infant mortality in the presence of this disease is insignificant.

Forecast

When a child with a severe form of lamellar ichthyosis is born, the probability of survival is about 20%, but if he has an initial stage of the disease, the survival rate rises.The appearance of manifestations of the disease in the adult state can very rarely become a risk to life.