Stevens-Johnson syndrome is an acute bullous lesion of the mucous membranes and skin of an allergic nature. It occurs against the background of a serious condition of the patient with the involvement of the oral mucosa, eyes and genitourinary organs. The diagnosis of Stevens-Johnson syndrome includes a thorough examination of the patient, an immunological blood test, a skin biopsy, and a coagulogram. According to the indications, lung radiography, ultrasound of the bladder, ultrasound of the kidneys, biochemical analysis of urine, consultations of other specialists are carried out. Treatment is carried out by methods of extracorporeal hemocorrection, glucocorticoid and infusion therapy, antibacterial drugs.
ICD 10
L51.1 Erythema bullosa multiforme
General information
Data on Stevens-Johnson syndrome were published in 1922. Over time, the syndrome was named after the authors who first described it. The disease is a severe variant of multiform exudative erythema and has a second name — “malignant exudative erythema”. Together with Lyell’s syndrome, pemphigus, bullous variant of SLE, allergic contact dermatitis, Hayley-Hayley disease, etc., clinical dermatology refers Stevens-Johnson syndrome to bullous dermatitis, the common clinical symptom of which is the formation of blisters on the skin and mucous membranes.
Stevens-Johnson syndrome is observed at any age, most often in people 20-40 years old and extremely rarely in the first 3 years of a child’s life. According to various data, the prevalence of the syndrome per 1 million population ranges from 0.4 to 6 cases per year. Most authors note a higher incidence among men.
Causes
The development of Stevens-Johnson syndrome is caused by an immediate allergic reaction. There are 4 groups of factors that can provoke the onset of the disease: infectious agents, medications, malignant diseases and undetermined causes.
In childhood, Stevens-Johnson syndrome occurs more often against the background of viral diseases: herpes simplex, viral hepatitis, adenovirus infection, measles, influenza, chickenpox, mumps. The provoking factor may be bacterial (salmonellosis, tuberculosis, yersiniosis, gonorrhea, mycoplasmosis, tularemia, brucellosis) and fungal (coccidiomycosis, histoplasmosis, trichophytia) infections.
In adults, Stevens-Johnson syndrome is usually caused by the administration of medications or a malignant process. Of the medications, the role of the causal factor is primarily assigned to antibiotics, nonsteroidal anti-inflammatory drugs, CNS regulators and sulfonamides. Lymphomas and carcinomas play a leading role among oncological diseases in the development of Stevens-Johnson syndrome. If it is not possible to establish a specific etiological factor of the disease, then they talk about the ideopathic Stevens-Johnson syndrome.
Symptoms
Stevens-Johnson syndrome is characterized by an acute onset with a rapid development of symptoms. At the beginning, malaise is noted, the temperature rises to 40 ° C, headache, tachycardia, arthralgia and muscle pain. The patient may be concerned about sore throat, cough, diarrhea and vomiting. After a few hours (maximum in a day), the appearance of rather large bubbles is observed on the oral mucosa. After their opening, extensive defects are formed on the mucosa, covered with white-gray or yellowish films and crusts of dried blood. The red border of the lips is involved in the pathological process. Due to severe mucosal damage in Stevens-Johnson syndrome, patients cannot eat or even drink.
Eye damage at the beginning proceeds according to the type of allergic conjunctivitis, but is often complicated by secondary infection with the development of purulent inflammation. For Stevens-Johnson syndrome, the formation of erosive and ulcerative elements of small size on the conjunctiva and cornea is typical. Possible damage to the iris, the development of blepharitis, iridocyclitis, keratitis.
The defeat of the mucosa of the genitourinary system is observed in half of the cases of Stevens-Johnson syndrome. It occurs in the form of urethritis, balanoposthitis, vulvitis, vaginitis. Scarring of erosions and ulcers of the mucous membrane can lead to the formation of urethral stricture.
The skin lesion is represented by a large number of rounded towering elements resembling blisters. They have a purple color and reach a size of 3-5 cm . A feature of the elements of skin rash in Stevens-Johnson syndrome is the appearance of serous or bloody blisters in their center. The opening of the bubbles leads to the formation of bright red defects, covered with crusts. The favorite localization of the rash is the skin of the trunk and perineum.
The period of appearance of new rashes of Stevens-Johnson syndrome lasts about 2-3 weeks, the healing of ulcers occurs within 1.5 months. The disease can be complicated by bleeding from the bladder, pneumonia, bronchiolitis, colitis, acute renal failure, secondary bacterial infection, loss of vision. As a result of the developed complications, about 10% of patients with Stevens-Johnson syndrome die.
Diagnostics
A dermatologist can diagnose Stevens-Johnson syndrome on the basis of characteristic symptoms detected by a thorough dermatological examination. The patient’s survey allows us to determine the causal factor that caused the development of the disease. A skin biopsy helps to confirm the diagnosis of Stevens-Johnson syndrome. Histological examination shows necrosis of epidermal cells, perivascular infiltration by lymphocytes, subepidermal blistering.
In a clinical blood test, nonspecific signs of inflammation are determined, a coagulogram reveals clotting disorders, and a biochemical blood test shows a reduced protein content. The most valuable in terms of the diagnosis of Stevens-Johnson syndrome is an immunological blood test, which detects a significant increase in T-lymphocytes and specific antibodies.
Diagnosis of complications of Stevens-Johnson syndrome may require back-seeding of separable erosions, coprogram, biochemical urine analysis, Zimnitsky test, ultrasound and CT of the kidneys, ultrasound of the bladder, lung x-ray, etc. If necessary, the patient is consulted by narrow specialists: an oculist, a urologist, a nephrologist, a pulmonologist.
It is necessary to differentiate Stevens-Johnson syndrome with dermatitis, for which the formation of blisters is typical: allergic and simple contact dermatitis, actinic dermatitis, During’s herpetiform dermatitis, various forms of pemphigus (true, vulgar, vegetative, leafy), Lyell’s syndrome, etc.
Treatment
Therapy is carried out with high doses of glucocorticoid hormones. Due to the defeat of the oral mucosa, the administration of drugs often has to be carried out by injection. Gradual dose reduction begins only after the symptoms of the disease subside and the general condition of the patient improves.
Extracorporeal hemocorrection methods are used to purify blood from immune complexes formed in Stevens-Johnson syndrome: cascade plasma filtration, membrane plasmapheresis, hemosorption and immunosorption. Plasma and protein solutions are transfused. It is important to introduce a sufficient amount of fluid into the patient’s body and maintain normal daily diuresis. Calcium and potassium preparations are used as additional therapy. Prevention and treatment of secondary infection is carried out with the help of local and systemic antibacterial drugs.
