Lyell syndrome

Lyell syndrome's disease overview

What is Lyell syndrome ? Lyell syndrome is also known as epidermal necrotic syndrome due to poisoning. This is a syndrome of many skin symptoms, mucosa and internal organs, onset is the contaminated body of the contaminated body, which are the contaminated red -body redders. Then, on the damaged skin appears large bullies leading to necrosis of large patches of epidermis such as high temperature burns.

The cause of Lyell syndrome is mostly due to drug allergies and this is the most severe clinical form of drug allergies. The prognosis of Lyell syndrome is very severe, the mortality rate is quite high, the cause of death is often due to ineffective treatment from the beginning. According to statistics, in the US accounts for about 35%, in Vietnam is more than 50% of the cases. Lyell syndrome onset as Stevens - Johnson syndrome, however, the lesions in the niche naturally spread throughout the body with peeled skin.

Lyell syndrome is different from other allergens caused by other pathogen sources. Regarding the pathology, most allergies are characterized by the direct reaction of the body to allergens. Lyell syndrome occurs when the protein that causes allergies is present in the cells of the epidermis, making the top layer of the skin become a "strange" object to the body and leads to the body that does not accept it. As a result, the body will quickly decompose strange substances, thus destroying the whole epidermis of the body. Allergic substances depend on each individual. Even harmless drugs can create a strong allergic reaction in some people.

Causes of Lyell syndrome's disease

The cause of allergies can be any substance that enters the body can go through one or more paths such as eating, drinking, applying, injecting, inhaling. The drugs play an important role in the appearance of any allergic disease including Lyell syndrome. Most cases are due to drugs (77% due to drugs, 23% spontaneous). Many researchers believe that antibacterial agent is the cause of the first allergic reaction. However, allergies can be activated by any drug. This is usually because a person's body increases sensitivity to those substances. Lyell syndrome usually appears in normal people, after using some drugs from 10 to 30 days, the earliest case is 1 day, the average is 14 days and there are cases up to 4o days. . Mostly, cases of lyell syndrome often use more than one drug, some people use 4-5 different drugs.

In nonsteroidal anti -inflammatory drugs that account for the highest proportion (about 43%), followed by sulfamid, especially slow sulfamid, accounting for 25%, 10%anti -convulsions, other drugs (Herpes resistance, hydrogenoine, haloperidol, anti -tuberculosis) 4%.

In addition, Lyell syndrome can also occur due to infection, vaccination, serum. Some cases of unknown reasons (IDIopathique).

Symptoms of Lyell syndrome's disease

The body's reaction with a strange substance in Lyell syndrome is quite fast, usually within a few days. Initially, the body temperature increased suddenly, then the rash covered the entire body. After only a few days, the skin forms blisters, like the image of the skin after a deep burn. The epidermis is peeling with some places on the body with lots of bleeding.

In addition to skin lesions, Lyell syndrome also causes mucosal damage. Mucosa lesions are not only in the mucosa in external organs such as the lip mucosa, eye mucosa, the mucosa of the genital organs but also damage the mucosa in the internal organs. The damaged mucosa leads to inflammation and peeling. The body temperature increases very quickly, leading to dehydration, characterized by weakness, drowsiness, disorientation. Losing a lot of bloody water makes it difficult for blood circulation in the vessel to then interrupting blood supply to important organs such as heart, kidneys, liver.

Clinically many cases of acute progress in a few hours, sometimes suddenly appears high fever, cold tremor, brushing chest drum, body weakness, insomnia, persistent mild headache, ulcerative pain The mouth, not delicious, muscle pain and myastal. In some mild cases, progressing into 2-3 days with a very typical clinical scene, sometimes coma, patients with high fever continuously 39 - 40 0C. 

In general, the signs for mild to heavy allergy cases may be:

Skin damage:

  • Red plates like measles or spreading rash.

    • Diverse roses.

  • Puffiness is like a thermal burn. The lesions quickly spread, dark red, slipped skin. Patients with hot, burning, nikolsky (+) signs.

    • Mucus lesions:

  • Puscular, conjunctivitis.
  • corneal ulcer is usually available in the eyes.
  • Red swelling, eye edema, difficult to open eyes.
  • Fear of light due to pain.
  • mouth ulcer, mouth mucosa, throat ulcer.
  • ulcerative esophageal, stomach, duodenal, intestinal mucosa.
  • ulcerative vaginal mucosa, vulva.

    • Systemic signs:

  • Fever: 39 - 40 0C (Almost always).
  • People are tired, sell coma even coma.

    • Gastrointestinal hemorrhage may be light or heavy.

  • Pneumonia, bronchitis, acute pulmonary edema.

    • CREATININ, URE ...

  • Hepatitis (increased enamel enzyme).
  • Damage at hematopoietic organs: white blood cells, platelets.

    • Acute pancreatitis.

    Water and electrolyte disorders.

    People at risk for Lyell syndrome's disease

    Babies began to face many environmental pathogens. These factors can cause many other diseases for children. However, the immunity from the mother will protect babies, so serious allergies such as Lyell syndrome are very rare in babies.

    Most cases of epidermal necrosis in adults often appear after medication, sometimes after blood transfusion and components of blood and serum. For children, Lyell syndrome often develops in young children due to staphylococcus infections. The cause is usually the presence of available genetic allergies.

    Prevention of Lyell syndrome's disease

    Lyell syndrome depends on the atopic allergy of each person, so the prevention is not always prevention. However, when avoiding exposure to diseased allergens completely does not appear. So for people with a history of allergies, it is necessary to avoid allergies, especially when allergies to drugs. Need to take carefully with allergens to ensure no contact.

    Diagnostic measures for Lyell syndrome's disease

    Diagnosis of Lyell syndrome can mostly rely on clinical signs, it is important to diagnose the disease early to apply timely and positive treatments against severe signs of severe signs. Disease:

    Diagnosis is based on the following factors:

  • There is a history of exposure to allergens to pay attention to the history of the use of drugs.
  • Million eggs on the skin and mucosa: puffiness, blistering on red skin, with acute and widespread removal of epidermis. Nikolsky signs.

    • Skin damage accompanied by mucosal lesions: oral mucosa, eyes, genitals.

  • Signs of damage to internal organs: Hematoma, liver, kidney, lung.

    • Lyell syndrome needs to be diagnosed with the following diseases:

  • Diverse roses
  • Parking skin poisoning
  • Staphylococcus aureus (ESA) is mainly seen in children under 5 years old

    • Kawasaki syndrome (Skin syndrome - mucosa - lymphadenopathy).

    Lyell syndrome's disease treatments

    As soon as the first signs of Lyell syndrome need to be hospitalized immediately. With Lyell syndrome, timely care plays a prerequisite in saving patients. The treatment of Lyell syndrome is the main purpose of fighting poisoning. Moreover, it is essential for treatment of water balance and electrolytes, ensuring blood circulation and functions of important organs.

    When Lyell syndrome is very severe, the patient needs to be treated by dialysis. The dialysis has two purposes. The first is to reduce the concentration of allergens and toxins and the second is normalization of the immune system, in short to help promote the recovery process.

    The general principle
  • First of all, the most important thing is to stop using allergic drugs.
  • Comprehensively evaluate patients for specific treatment plans.
  • Evaluation of life function and necessary tests.

    • To assess the severity and prognosis, use the Scorten scale. specific treatment on the spot:

  • Treatment at the emergency room, ensuring aseptic.

    • Skin care such as treating severe burns: Patients lying on beds spreading talc powder.

  • Wash the daily damaged skin areas with physiological saline or dilute purple medicine 1/5,000-1/10,000. Can apply or wrap the gauze bandage with antibiotic.
  • Mucosa: Daily hygiene with physiological saline, applying oral mucosa with Glycerin Borat solution, regular mouthwash with diluted hydrated oxygen water, applying kamistad Gel
  • Eye lesions: antibiotic ointment, vitamin A oil, peeling eyelids to avoid inflammation, conjunctivitis, eyelids, blind eyes. >
    • Systemic treatment
  • Diet: Need to eat liquid, cool, ensure adequate nutrition, lots of protein are milk, nutritious porridge, eat a lot Meals, necessary to eat via sonde.
  • Fertilizer transmission or fresh plasma
  • Support and electrolyte according to the specific situation of patients
  • Pain relievers

    • Antihistamine resistance antibiotic: Often use broad -spectrum antibiotics, less allergic to prevent skin, lung infections, blood infections.

  • corticosteroids: In case of indications, one of the following two ways: The dose from 1-2 mg/kg of weight, sometimes up to 4mg/kg of weight, dose 100 -250 mg intravenously in the first 3-4 days. When skin and body damage progresses well, it is possible to consider reducing the dose to avoid drug complications.

    • Globulin immunity with high doses of intravenously, 1mg/kg weighs 3 days. In addition, it is necessary to treat complications in the liver, kidneys, water and electrolytes, gastrointestinal bleeding, esophageal and vaginal narrowing.

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