Stevens-Johnson Syndrome

Stevens-Johnson syndrome is a toxic-allergic disease that is a malignant variant of bullous multiforme exudative erythema.

[1], [2], [3], [4], [5]

Causes of the stevens-Johnson syndrome

Possible causes of the disease are often medicines (antibiotics, sulfonamides, analgesics, barbiturates, etc.). The disease can occur on other allergens. Currently, most dermatologists believe that the basis of multiform exudative erythema and Stevens-Johnson syndrome and Lyell's syndrome is a toxic-allergic reaction. Clinically and pathogenetically, the difference between them is substandard, but quantitative. In this case, the antigen-antibody reaction is directed to keratinocytes with the formation of circulating immune complexes in the blood serum, the deposition of the IgM and S3 complement component along the epidermal basement membrane and the upper dermis.

[6], [7], [8], [9],

Symptoms of the stevens-Johnson syndrome

The disease is characterized by a violent onset with pronounced common phenomena (high fever, arthralgia, myalgia). Then after a few hours or 2-3 days, there are lesions of the skin and mucous membranes.

On the skin of the trunk, upper and lower extremities, the face, genital organs appear disseminated round erythema-swollen spots of crimson color with a peripheral zone and sunken cyanotic center with a diameter from 1 to 3-51 cm. Such elements of the rash resemble eruptions of multiforme exudative erythema. Then in the center of many of them thin-walled flaccid bubbles with serous or hemorrhagic contents are formed. Bubbles, merging, reach huge sizes. Opening up, they leave juicy bright red painful erosions, along the edges of which there are scraps of bubble covers ("epidermal collar"). Under the influence of light touch, the epidermis "slides" (a positive symptom of Nikolsky). The surface of erosion over time is covered with yellowish-brown or hemorrhagic crusts.

On the mucosa of the mouth, eyes are marked with hyperemia, edema, appear flaccid bubbles, after the opening of which the formation of painful large erosions. Red lip rim is sharply edematous, hyperemic, has bleeding cracks and is covered with crusts. Often there are phenomena of blepharoconjunctivitis up to panophthalmia, lesions of the urethral mucosa, bladder, upper respiratory tract. Severe common phenomena (fever, malaise, headaches, etc.) continue for 2-3 weeks.

Complications and consequences

Against this background, often develop pneumonia, diarrhea, kidney failure and other complications. In 30% of cases, a lethal outcome is observed.

[10], [11], [12], [13], [14]

Diagnostics of the stevens-Johnson syndrome

In the blood leukocytosis, eosinopenia, increased ESR, increased bilirubin, urea, aminotransferases, changes in fibrinolytic activity of the plasma due to activation of the proteolytic system, decrease in the total amount of protein due to albumins.

[15], [16], [17], [18], [19]

Differential diagnosis

Differential diagnosis is performed with pemphigus, Lyell syndrome, etc.

[20]

Treatment of the stevens-Johnson syndrome

Corticosteroids are prescribed at the rate of 1 mg / kg of the weight of the patient (in terms of prednisolone) until a pronounced clinical effect is achieved, followed by a gradual decrease in the dose until complete withdrawal within 3-4 weeks. If oral administration is not possible, corticosteroids should be administered parenterally. There are also activities aimed at inactivating and removing antigens and circulating immune complexes from the body, using enterosorbents, hemosorption, plasmapheresis. To combat the syndrome of endogenous intoxication, 2-3 liters of liquid per day are administered parenterally (saline, hemodez, Ringer's solution, etc.), as well as albumin and plasma. Assign also preparations of calcium, potassium, antihistamines, in case of the threat of infection - antibiotic a wide range. Outwardly, corticosteroid creams (Lorinden C, dermovit, advantan, triderm, celostidm with garomycin, etc.), 2% aqueous solutions of methylene blue, gentiiaviolet are used.