Lyell's Syndrome: causes, symptoms, treatment, prognosis

August 21, 2017 23:00 | Allergies

As a serious skin lesion with characteristic manifestations, Lyell's syndrome( as a form of toxicodermia) requires rapid intervention to prevent serious consequences and save the patient's life.Having many reasons that can cause the onset of this skin condition, Lyell's syndrome even at the initial stages of development is accompanied by abrupt changes in the condition of the skin that attracts attention and allows you to diagnose the disease in time.

With several synonym names, Lyell's syndrome can occur at almost any age.However, according to medical statistics, the most common pathological condition of the skin is diagnosed in the elderly: from 45 to 68 years, when the defenses of the body are weakened, there are a number of diseases with a chronic course.This article will describe the full clinical picture of Lyell's syndrome and its pathogenesis.

Features of the disease

Lyell syndrome can occur in both women and men.However, more often from the male half of the population with characteristic ex

ternal manifestations and constantly worsening the poor state of health of the patient.

Along with the manifestations of severe skin lesions and exfoliation of the upper layer of the epidermis, mucosal lesions are noted. The most susceptible to negative fusion of this pathology is the mucous membrane of the mouth, larynx, and genital organs.

Toxic epidermal necrolysis - Lyell's syndrome( photo)

Classification

Depending on the stage of the current process, the disease can go through several stages.

At the initial stage of the lesion, an increase in body temperature is observed, which is accompanied by deterioration of the general condition, the appearance of drowsiness and loss of orientation in space.Along with this, at the first stage of the development of the disease on the skin, lesions begin to appear that are characterized by reddening of the skin areas, an increase in its sensitivity.

In the second stage of disease progression, the areas with affected skin increase in size, the hypersensitivity of the skin also increases.The skin begins to separate, bubbles with serous contents form on its surface, the thirst appears in the patient, the production of saliva decreases.

The third stage is characterized by a general weakening of the patient's body, there are all signs of dehydration.In the absence of medical care or its insufficiency, a fatal outcome is likely.

The reasons for the occurrence of Lyell's syndrome are described in the video below:

Causes of

The occurrence of this disease can be associated with several causes, the most common of which is the body's tendency to manifest allergic reactions.It can be hereditary or acquired factor.

Also to the reasons that can cause the emergence of a condition such as Lyell's syndrome, we can refer:

  • infectious diseases of the body that dramatically reduce the degree of efficiency of the immune system;
  • use of certain medications, one of which contained sulfidamines;
  • affection of the body with staphylococci, which is most characteristic for childhood;
  • An idiopathic variant of the disease can develop due to psychological overstrain, nervous overload and depressed states of prolonged course.

Also to the reasons for the development of the Lyell syndrome are a combination of the listed reasons or a combination thereof.

Symptoms of Lyell's syndrome in children and adults, its diagnosis and treatment are described below.

Symptoms

The most characteristic manifestations of this disease include deterioration of the skin.In this case, the following symptoms are noted:

  • body temperature rise up to 39-40 °, while in some parts of the body spots with brightly colored skin are formed.The size of such sites can vary significantly, their location is different.Spots can be single, then they begin to merge;
  • after a few hours( 10-12) on the surface of such spots, swellings form, the upper layer of the epidermis begins to flake off.Bubbles resemble burns of the 2nd degree, because the disease has the second name "malignant pemphigus";
  • gradually the process covers an ever larger skin surface, in parallel there is a significant deterioration in the patient's condition.

When opening formed bubbles, exudate serous-bloody composition is allocated, as a result of which rapid dehydration of the patient is observed.After opening the blisters on the skin, more erosion remains, the skin on them has a bright red color and increased sensitivity.

If Lyell's syndrome is detected, the current condition gradually worsens, the skin surface begins to separate, even with a slight mechanical effect, epidermis is segregated to form significant erosions, with no bubbles forming on the skin.Body temperature continues to be elevated.

High degree of soreness, tendency to aggravation of pathological process and poor state of health - these manifestations also accompany the main symptomatology of the current illness.

The following describes the diagnosis of Lyell's syndrome.

Diagnostics

Diagnosis allows you to identify a beginning disease in the earliest stages, which allows you to quickly arrest the pathological process.Conducting an external examination of the patient, his skin and conducting a number of laboratory tests allows you to determine most accurately both the defeat itself and the current stage.On the basis of the obtained data, the scheme of the therapeutic effect is determined.

The methods of diagnostic study include:

  • biochemical and general blood test,
  • urine analysis,
  • coagulogram data,
  • and biopsy of skin particles of the affected person.

Treatment, emergency care and clinical recommendations for patients with Lyell's syndrome are given below.

Treatment

The effect on the skin in the initial stages of the disease can be carried out both therapeutic and medicinal method. First of all, any chosen treatment is aimed at removing pain manifestations, eliminating the hypersensitivity of the skin.

Therapeutic method

  • For the removal of pain at the beginning of the development of the pathological process, it may be recommended to moisten the affected areas with cold water, which also facilitates the general condition at elevated body temperature.
  • However, due to the activity of the process with the rapid progression of the disease and the deterioration of the patient's condition, emergency care and the receipt of certain drugs with high efficacy are required.

When diagnosing Lyell's syndrome, the victim should be assisted in the intensive care unit of a medical institution.The fastest use of funds that remove the soreness of the skin, eliminating the active pathological process with the stratification of the upper layer of the epidermis, allows to stabilize the patient's condition.

Large doses of injectable corticosteroids, painkillers and the use of hemocorrection methods can stop the current process.With marked improvement, all medications used are given to the patient in a reduced amount.

In the treatment of this disease, an integrated approach is used combining the use of antibacterial drugs of the latest generation of a wide spectrum of action, agents that stimulate and support the kidneys, as well as means for reducing blood coagulability.

Other methods

  • Surgical intervention in detecting Lyell's syndrome is usually not required.
  • However, along with the medicinal effect should regularly performed dressings, the change of underwear to sterile, treatment of not only damaged skin, but also mucous membranes.

Prevention of Lyell's syndrome

To prevent the onset of this pathological condition of the skin, it is necessary to exclude the treatment of any disease by several drugs at the same time( no more than 5-7 agents at a time), with a tendency to allergic manifestations to exclude self-treatment and in any abnormalitySeek help from a doctor.

Complications

Due to the rapid development of the current process, the likelihood of negative manifestations in the functioning of the kidneys is high, therefore maintenance therapy allows to avoid the possibility of negative manifestations.Also, if the treatment is inadequate, skin deterioration may occur.

The prognosis of the statistics of those who survived with Lyell's syndrome is described below.

Forecast

In the provision of timely care, life expectancy decreases slightly:

  • The 5-year survival rate for the treatment of the disease in the initial stage is 95-98%,
  • with the development of the disease and its treatment at a later stage, index 5Year survival rate is about 75-82%.