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

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

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

[1], [2]

What causes Lyell's syndrome?

The causes of Lyell's syndrome are the effects of medications. Among them, the first place is occupied by sulfanilamide preparations, followed by antibiotics, anti-convulsants, anti-inflammatory and anti-tuberculosis drugs. The period of development of Lyell's syndrome from the moment of taking the medication that caused it varies from a few hours to 6-7 days. An important place is occupied by hereditary factors. 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 of NAS-A2, A29, B12, and Dr7 has been found.

At the heart of the disease are toxic-allergic reactions in both multiforme exudative erythema and Stevens-Jopson syndrome.

Histopathology of Lyell's Syndrome

Necrosis of the surface layers of the epidermis is observed. The cells of the malpighian layer are edematous, the intercellular and epidermodermal connections are broken (exfoliation of the epidermis from the dermis). As a result, blisters are formed on the skin and mucous membranes, which are located inside and subepithelial.

Pathology of Lyell's Syndrome

In the region of the bubble, located subepidermally, necrobiotic changes in the epidermis of necrolysis type are revealed, which are expressed in the complete loss of the structure of its layers. As a result of disturbance of intercellular contacts and hydrophytic dystrophy of individual cells of the epidermis, ballooning cells with pycnotic nuclei appear. Between the destructively altered cells there are many neutrophilic granulocytes. In the cavity of the bladder - balloon cells, lymphocytes, neutrophil granulonites. In the dermis there is swelling and small infiltrates from the 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 the deeper parts are fragmented.

In clinically unchanged skin with Lyell's syndrome, separation of small areas of the stratum corneum and, in some places, total epidermis, is observed. Basal layer cells are mostly pycnotic, some of them are vacuolated, the intervals between them are enlarged, sometimes filled with homogeneous masses. In some papillae of the dermis fibrinoid swelling is expressed. The endothelium of the vessels is swollen, around some of them small lymphocytic infiltrates are noted.

Symptoms of Lyell's Syndrome

According to the research, 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. They attach importance to the development of the process and staphylococcal infection. A.A. Kalamkaryan and V.A. Samsonov (1980) regards Lyell's syndrome as a synonym for bullous multiforme exudative erythema and Stevenson-Johnson syndrome. Probably, this identification can be only in relation to the secondary form of the exudative multiformin erythema caused by medications. OK Steigleder (1975) identifies three variants of Lyell's syndrome: epidermal necrolysis caused by staphylococcal infection identical to Ritter's exfoliative dermatitis, and in adults - to Rittersgain-Lyell syndrome; epidermal necrolysis caused by drugs; the most difficult idiopathic variant of an unclear etiology. Clinically, the disease is characterized by a general severe condition of patients, high fever, multiple erythematopoietic and bullous eruptions reminiscent of those with pemphigus, with eccentric growth, rapidly opening up to form extensive painful erosions similar to skin lesions in burns. Nikolsky's symptom is positive. Affects mucous membranes, conjunctiva, genitalia. It is observed more often in women, often occurs in children.

The disease Lyell's syndrome develops after several hours or days after the first intake of the drug. But not in all cases, you can see the relationship of the disease with the previous intake of medications. In the clinical picture of the Lyell syndrome, as in the Stevens-Jopson syndrome, the skin and mucous membranes are lesioned on the foyer of a severe general condition. The most characteristic lesion of the mucous membranes of the mouth, nose in the form of hyperemia, edema followed by the development of large blisters, which, rapidly opening, form extremely painful extensive erosion and ulceration with the remnants of the blister covers. In some cases, the inflammatory process extends to the mucous membrane of the pharynx, gastrointestinal tract, larynx, trachea and bronchi, causes severe attacks of dyspnea. There is stagnant-cyanotic hyperemia and swelling of the skin, conjunctiva of the eyelids with the formation of blisters and erosions, covered with serous-bloody crusts. The lips are swollen, covered with hemorrhagic crusts. Because of severe pain, even liquid food is difficult to take. 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, a rash appears that consists of erythematous-swollen spots or crimson-red elements that merge into large plaques. Elements are typical for multiform exudative erythema and Stevens-Johnson syndrome. Quickly formed flat flabby bubbles with scalloped contours, the size to the palm of an adult and more, filled with serous and serous-bloody fluid. Bubbles under the influence of minor injuries are opened and extensive wet, sharply painful erosive surfaces with scraps of epidermis on the periphery are formed. The symptom of Nikolsky is sharply positive: at the slightest touch, the epidermis is easily separated on a vast stretch ("the symptom of the sheet"). Extensive eroded lesions on the back, abdomen, buttocks, thighs resemble the skin with a second degree burn. On the palms, it is separated by large plates like a glove. The general condition of patients is extremely severe: high temperature, prostration, dehydration symptoms are often accompanied by dystrophic changes in internal organs. In many cases, moderate leukocytosis with a shift to the left is detected. In 40% of cases, patients quickly die.

Diagnosis of Lyell's Syndrome

As indicated, the development of the disease is most often associated with the use of medications, possibly possessing epidermotropism. An association of the disease with certain 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 are data on the possible role of immune complexes in its pathogenesis.

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

[3], [4], [5], [6]

Treatment of Lyell's Syndrome

It is necessary to use early daily large doses of corticosteroids (1-2 mg / kg of the patient's weight), antibiotics, vitamins. Detoxification measures - the introduction of plasma, blood substitutes, liquids, electrolytes; sterile underwear. Often patients who have Lyell syndrome need resuscitation.

What is the prognosis of Lyell's syndrome?

The Lyell syndrome has an unfavorable prognosis. Fatal outcomes are observed in about 25% of patients.