Lyell's syndrome: causes, symptoms, diagnosis, treatment

Lyell's syndrome (synonyms: acute epidermal necrolysis, toxic epidermal necrolysis, scalded skin syndrome) is a severe toxic-allergic disease that threatens the patient's life, characterized by intense detachment and necrosis of the epidermis with the formation of extensive blisters and erosions on the skin and mucous membranes, a toxic-allergic disease, the main cause of which is increased sensitivity to drugs (bullous drug disease).

Many believe that Stevens-Johnson syndrome and Lyell's syndrome are severe forms of erythema multiforme exudative, and their clinical differences depend on the severity of the pathological process.

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What causes Lyell's syndrome?

The causes of Lyell's syndrome are the effects of drugs. Among them, sulfonamides take the first place, followed by antibiotics, anticonvulsants, anti-inflammatory and anti-tuberculosis drugs. The period of development of Lyell's syndrome from the moment of starting to take the drug that caused it varies from several hours to 6-7 days. Hereditary factors play an important role. Thus, approximately 10% of the human population has a genetic predisposition to allergic sensitization of the body. An association of Lyell's syndrome with antigens HAH-A2, A29, B12, Dr7 has been discovered.

The disease is based on toxic-allergic reactions, as in erythema multiforme exudative and Stevens-Jopson syndrome.

Histopathology of Lyell's syndrome

Necrosis of the superficial layers of the epidermis is observed. The cells of the Malpighian layer are edematous, the intercellular and epidermodermal connection is disrupted (the epidermis separates from the dermis). As a result, blisters form on the skin and mucous membranes, which are located inside and subepithelially.

Pathomorphology of Lyell's syndrome

In the area of the blister, located subepidermally, necrobiotic changes in the epidermis of the necrolysis type are found, expressed in the complete loss of the structure of its layers. As a result of the disruption of intercellular contacts and hydropic dystrophy of individual epidermal cells, ballooning cells with pyknotic nuclei appear. There are many neutrophilic granulocytes between the destructively changed cells. In the cavity of the blister - ballooning cells, lymphocytes, neutrophilic granulonites. In the dermis - edema and small infiltrates of lymphocytes. In the area of skin defects, the upper layers of the dermis are necrotic, thrombosed vessels and focal lymphocytic infiltrates are revealed. Collagen fibers of the upper third of the dermis are loosened, and in deeper parts they are fragmented.

In clinically unchanged skin with Lyell's syndrome, separation of small areas of the stratum corneum and, in places, the entire epidermis is observed. The cells of the basal layer are mostly pycnotic, some of them are vacuolated, the spaces between them are widened, sometimes filled with homogeneous masses. Fibrinoid swelling is expressed in some dermal papillae. The vascular endothelium is swollen, and small lymphocytic infiltrates are noted around some of them.

Symptoms of Lyell's syndrome

According to research data, only 1/3 of patients with this disease did not receive medications, and most of them took more than one name. Most often, these are sulfonamides, especially sulfamethoxazone and trimethoprim, anticonvulsants, primarily phenylbutazone derivatives. Staphylococcal infection is also considered important in the development of the process. A.A. Kalamkaryan and V.A. Samsonov (1980) consider Lyell's syndrome as a synonym for bullous multiform exudative erythema and Stevenson-Johnson syndrome. Probably, this identification can only be in relation to the secondary form of exudative erythema multiforme caused by drugs. OK Steigleder (1975) identifies three variants of Lyell's syndrome: epidermal necrolysis caused by staphylococcal infection, identical to Ritter's exfoliative dermatitis of the newborn, and in adults - Rittersheim-Lyell syndrome; epidermal necrolysis caused by drugs; the most severe idiopathic variant of unclear etiology. Clinically, the disease is characterized by a general severe condition of patients, high temperature, multiple erythematous-edematous and bullous rashes resembling those in pemphigus, with eccentric growth, quickly opening with the formation of extensive painful erosions similar to skin lesions in burns. Nikolsky's symptom is positive. Mucous membranes, conjunctiva, genitals are affected. It is observed more often in women, often occurs in children.

Lyell's syndrome develops several hours or days after the first intake of a drug. But not in all cases can one notice a connection between the disease and previous drug intake. In the clinical picture of Lyell's syndrome, as in Stevens-Jopson syndrome, there is damage to the skin and mucous membranes in the background of a severe general condition. The most typical is damage to the mucous membranes of the mouth, nose in the form of hyperemia, edema with subsequent development of large blisters, which, quickly opening, form extremely painful extensive erosions and ulcers with remnants of the blister caps. In some cases, the inflammatory process spreads to the mucous membrane of the pharynx, gastrointestinal tract, larynx, trachea and bronchi, causing severe attacks of dyspnea. Congestive-cyanotic hyperemia and edema of the skin, conjunctiva of the eyelids with the formation of blisters and erosions covered with serous-bloody crusts are observed. The lips are swollen and covered with hemorrhagic crusts. Due to the sharp pain, it is difficult to take even liquid food. A rash consisting of erythematous-edematous spots or elements of a purple-red color, which merge to form large plaques, suddenly appears on the skin of the trunk, extensor surfaces of the upper and lower extremities, as well as on the face and in the genital area. The elements are typical of erythema multiforme exudative and Stevens-Johnson syndrome. Flat, flabby blisters with scalloped outlines, up to the size of an adult's palm or more, filled with serous and serous-bloody fluid, quickly form. Under the influence of minor injuries, the blisters open and extensive, weeping, sharply painful erosive surfaces with epidermal shreds along the periphery are formed. Nikolsky's symptom is sharply positive: with the slightest touch the epidermis easily separates over a large area ("sheet symptom"). Extensive eroded lesions on the back, abdomen, buttocks, thighs resemble the skin in a second-degree burn. On the palms it separates in large plates like a glove. The general condition of patients is extremely serious: high temperature, prostration, symptoms of dehydration are often accompanied by dystrophic changes in the internal organs. In many cases, moderate leukocytosis with a shift to the left is detected. In 40% of cases, patients die quickly.

Diagnosis of Lyell's syndrome

As indicated, the development of the disease is most often associated with the use of drugs that may have epidermotropism. An association of the disease with some tissue compatibility antigens, such as HLA-A2, HLA-A29, HLA-B12, HLA-DR7, has been found. The immune mechanism in acute toxic epidermolysis is unclear. There is evidence of a possible role of immune complexes in its pathogenesis.

Differential diagnosis should be made with pemphigus, exfoliative dermatitis of newborns.

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Treatment of Lyell's syndrome

It is necessary to administer large daily doses of corticosteroids (1-2 mg/kg of patient weight), antibiotics, and vitamins early. Detoxifying measures include administration of plasma, blood substitutes, fluids, electrolytes; sterile linen. Patients with Lyell's syndrome often require resuscitation care.

What is the prognosis for Lyell's syndrome?

Lyell's syndrome has a poor prognosis. Fatal outcomes occur in approximately 25% of patients.