Stevens johnson syndrome Treatment, Causes, Signs Diagnosis & Pictures: Stevens-Johnson Syndrome is an acute bullous lesion of mucous membranes and skin of an allergic nature. It proceeds against the background of the serious condition of the patient with the involvement of the oral mucosa, eyes and urinary organs. Diagnosis of Stevens-Johnson syndrome includes a thorough examination of the patient, an immunological study of blood, skin biopsy, coagulogram. According to indications, radiography of the lungs, ultrasound of the bladder, ultrasound of the kidneys, biochemical analysis of urine, consultation of other specialists. The treatment is carried out by methods of extracorporal hemocorrection, glucocorticoid and infusion therapy, antibacterial drugs.
Content in this article:
- Stevens Johnson Syndrome History
- Treatment of Stevens-Johnson syndrome
Stevens-Johnson Syndrome History
Data on Stevens-Johnson syndrome was published in 1922. Over time, the syndrome was named after the authors who first described it. The disease is a severe variant of exudative erythema multiforme and has a second name – “malignant exudative erythema”. Together with Lyell’s syndrome , pemphigus , a bullous variant of SLE, allergic contact dermatitis , Haley-Hayley’s disease , and others. Clinical dermatology classifies Stevens-Johnson syndrome to bullous dermatitis , the common clinical symptom of which is blistering on the skin and mucous membranes.
Stevens-Johnson syndrome is observed at any age, most often in people 20-40 years old and extremely rare 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 report a higher incidence among men.
Causes of Stevens-Johnson syndrome
The development of Stevens-Johnson syndrome is due to an immediate allergic reaction. There are 4 groups of factors that can trigger the onset of the disease: infectious agents, drugs, malignant diseases, and unknown causes.
In childhood, Stevens-Johnson syndrome occurs more frequently against the background of viral diseases: herpes simplex , viral hepatitis , adenovirus infection , measles, influenza , chicken pox, and epidemic parotitis . Bacterial ( salmonellosis , tuberculosis, yersiniosis , gonorrhea , mycoplasmosis, tularemia , brucellosis ) and fungal infections ( coccidiomycosis , histoplasmosis , trichophytosis ) infections can be a provoking factor .
In adults, Stevens-Johnson syndrome is usually caused by the administration of medications or a malignant process. Of the drugs, the role of the causative factor in the first place is given to antibiotics, nonsteroidal anti-inflammatory, regulators of the central nervous system and sulfonamides. The leading role among oncological diseases in the development of Stevens-Johnson syndrome is played by lymphomas and carcinomas. If the specific etiological factor of the disease cannot be established, then they talk about the idiopathic Stevens-Johnson syndrome.
Stevens-Johnson syndrome is characterized by an acute onset with rapid development of symptoms. At the beginning, there is malaise, a rise in temperature up to 40 ° C, headache, tachycardia , arthralgia and muscle pain. The patient may experience sore throat, cough, diarrhea, and vomiting. Within a few hours (maximum after 24 hours), rather large blisters appear on the oral mucosa. After opening them, extensive defects are formed on the mucous membrane, 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 lesions in Stevens-Johnson syndrome, patients cannot eat or even drink.
Damage to the eyes at the beginning of the type of allergic conjunctivitis , but is often complicated by secondary infection with the development of purulent inflammation. For Stevens-Johnson syndrome, education on the conjunctiva and cornea of erosive-ulcerative elements of small size is typical. Possible damage to the iris, the development of blepharitis , iridocyclitis , keratitis .
The lesion of the mucous membranes of the genitourinary system is observed in half the cases of Stevens-Johnson syndrome. It occurs in the form of urethritis , balanoposthitis , vulvitis , vaginitis . Scarring of erosions and ulcers of the mucous membranes can lead to the formation of a urethral stricture.
Skin lesions are 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 bubbles in their center. The opening of the bubbles leads to the formation of bright red defects that become crusted. The favorite localization of the rash is the skin of the trunk and perineum.
The period of appearance of new rash of Stevens-Johnson syndrome lasts about 2-3 weeks, healing of ulcers occurs within 1.5 months. The disease may 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.
The clinician-dermatologist can diagnose Stevens-Johnson syndrome on the basis of the characteristic symptoms detected during a thorough dermatological examination. Interviewing a patient can determine the causative factor that caused the development of the disease A skin biopsy helps confirm the diagnosis of Stevens-Johnson syndrome . Histological examination shows epidermal cell necrosis, perivascular lymphocyte infiltration, and subepidermal blistering.
In the clinical analysis of blood, non-specific signs of inflammation are determined, the coagulogram detects bleeding disorders, and the biochemical analysis of blood 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 baccosev removal of erosions, coprograms, biochemical analysis of urine, Zimnitsky sample, ultrasound and CT of the kidneys , ultrasound of the bladder, x-ray of the lung , etc. .
Differentiating Stevens-Johnson syndrome is necessary with dermatitis , which is typical for blistering: allergic and simple contact dermatitis , actinic dermatitis , herpetiform Dühring dermatitis , various forms of pemphigus (true, vulgar , vegetative , leafy ), Lyell’s syndrome, etc.
Treatment of Stevens-Johnson syndrome
Therapy is carried out with high doses of glucocorticoid hormones. In connection with the defeat of the oral mucosa, the administration of drugs is often necessary to carry out the injection method. A gradual reduction in dose begins only after the symptoms of the disease subside and the patient’s general condition improves.
Extracorporeal hemocorrection methods are used to purify blood from immune complexes formed in Stevens-Johnson syndrome : cascade filtration of plasma , membrane plasmapheresis , hemosorption, and immunosorption . Produced plasma transfusion and protein solutions. It is important to introduce into the patient a sufficient amount of fluid and maintain normal daily diuresis. As an additional therapy, calcium and potassium preparations are used. Prevention and treatment of secondary infection is carried out with the help of local and systemic antibacterial drugs.