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What is Stevens-Johnson Syndrome
Stevens Syndrome – Johnson – this is a pathological process of an allergic nature, affecting the mucous membranes of organs and skin. The disease is severe, affecting more men in young and middle age. A characteristic feature of the syndrome is the formation of blisters with the subsequent occurrence of erosion in their place.
The causes of the disease
The disease is manifested by acute bullous inflammation and has the nature of the occurrence of an allergic nature. Such a pathology is a response of the body to the introduction of certain allergens into it.
The pathogenesis of the occurrence of the disease to the end remains unclear. It is believed that during its development, there is an increase and stimulation of cytotoxic T - lymphocytes, which destroy skin cells. This pathological process leads to exfoliation of the epidermis from the dermis.
The following factors can provoke the development of malignant erythema, which can be combined into several groups:
- infectious agents;
- medicinal products;
- volumetric processes of a malignant nature;
- unexplained reasons.
In addition, the probability of pathogenesis of a genetic nature is considered, when the result of a malfunction in the body is a significant decrease in its protective forces. This process leads to pathological changes not only on the skin, but also on the membranes of the blood vessels.
Risk Factors for Syndrome
The development of the process in adult patients is mainly provoked by the following factors:
- malignant neoplasms;
- taking various medications.
Unlike adults, Stevens-Johnson syndrome in children can develop against the background of the following infections:
- measles;
- parotitis;
- ARVI;
- chicken pox.
In addition to viral pathology, the following pathologies play an important role in the occurrence of malignant exudative erythema:
- salmonellosis;
- tuberculosis;
- tularemia;
- gonorrhea;
- brucellosis;
- trichophytosis.
Sometimes the syndrome manifests itself as a result of ingestion of food allergens, various chemicals, or as an organism’s response to vaccine administration. It is not always possible to determine the cause of the development of pathology. In this case, an idiopathic form of the syndrome occurs, which occurs in a quarter of patients.
Symptoms and signs of the disease
A distinctive feature of pathology is the acute onset of the disease with subsequent progression and the presence of clinical manifestations:
- general weakness;
- rise in temperature to high rates;
- sore muscles and joints;
- tachycardia;
- dry cough, sore throat and sore throat;
- vomiting and loose stools.
The presence of symptoms similar to ARVI and the progression of the process require immediate medical attention and urgent early treatment, especially with an allergic mood in the body.
After the onset of the disease, the symptoms of the disease develop further, affecting the skin and mucous membranes:
- oral cavity - clinical manifestations begin to grow rapidly and within a day, significant bubbles appear on the mucous membrane. Opening of these formations is accompanied by an extensive erosive process. The erosion surface begins to become covered with films and blood crusts. This pathology can spread to the red border of the lips, which is sometimes an obstacle for the patient to take food and water;
- eye - at the initial manifestations of the disease, damage to the organ of vision is like conjunctivitis, which has an allergic mood. However, it often gives a complication in the form of infection and the development of a purulent process. On the conjunctiva and cornea of the eyes, the formation of erosions and ulcers of a small size, that is, keratitis develops. In addition, pathology can spread to the iris and eyelids, causing iridocyclitis and blepharitis;
- genitalia - a lesion that is observed in a significant part of cases of the pathological process. It occurs in the form of inflammatory phenomena with the formation of erosions and ulcers in the area of the urethra (urethritis), foreskin of the male genitalia (balanoposthitis), external female genitalia (vulvitis), and vagina (vaginitis). Often this process can result in the formation of a stricture, that is, a narrowing of the urethra;
- skin - the appearance of rashes on the limbs, chest and back, growing into blisters of significant size up to 0.5 cm in diameter, containing a transparent or blood secret inside. After opening, these formations turn into erosions and ulcers, covered with crusts.
The period of active rashes with Stevens-Johnson syndrome lasts up to 3 weeks, and the recovery process of healing of erosion and ulcers can be long and last for several months.
A disease is especially dangerous during pregnancy, which can result in miscarriage or premature birth due to the severity of the pathology and the development of high intoxication of the body of the expectant mother.
Classification of malignant exudative erythema
Currently, pathology has several conditional classifications that are distinguished by certain parameters.
By provoking factor:
- idiopathic type - the most common malignant erythema, the occurrence of which is provoked by viruses and fungi;
- symptomatic type - the occurrence of pathology - this is a consequence of the use of drugs or vaccination.
By the nature of the inflammatory process:
- mild form - skin rashes are insignificant and are not accompanied by a change in the general condition of the patient;
- severe form - occurs with severe clinical manifestations, accompanied by various complications that threaten the life of the patient.
By type of rash:
- vesicular erythema - the appearance on the skin of small blisters, with a diameter of up to 4 mm, filled with serous secretion;
- maculopapular erythema - the appearance on the skin of spots and papules without a secret inside the formations;
- bullous erythema - the appearance of blisters of large diameter (up to 2 cm) filled with serous secretion;
- vesicle - erythema bullosa - is characterized by the appearance of spotted rashes and blisters on the skin and mucous organs. It is characterized by a severe course.
Diagnostic measures
Early diagnosis of the rapid symptoms of Stevens-Johnson syndrome is of great importance. Timely prescribed treatment will stop the progression of the disease and prevent the development of complications.
Diagnosis of the disease is carried out on the basis of an anamnesis, objective examination, laboratory data, including:
- general blood analysis;
- blood chemistry;
- immunological blood test;
- coagulogram;
- skin biopsy;
- bacterial inoculation of erosion contents;
- general and biochemical analysis of urine;
- Zimnitsky test.
According to indications, an instrumental examination of organs is carried out:
- Ultrasound of the kidneys;
- CT or MRI of the kidneys;
- Ultrasound of the bladder;
- radiography of the lungs.
Differential diagnosis of malignant exudative erythema is carried out with various skin inflammatory processes, accompanied by the formation of blisters. This is an allergic and simple contact dermatitis, actinic and herpetiform dermatitis.
In addition, the syndrome is differentiated with various forms of pemphigus, as well as Lyell syndrome. Differential diagnosis is carried out on the basis of patient complaints, an anamnesis of the development of pathology, symptoms, laboratory parameters.
Stevens-Johnson Syndrome Treatment
Therapy of the disease is carried out comprehensively and is aimed at improving the well-being of the patient, stopping the elements of rashes, as well as preventing the development of complications.
Since the pathological process has a tendency to spread rapidly, therapeutic measures are carried out on an emergency basis in the following form:
- intravenous administration of hemodesis, glucose, plasma;
- extracorporeal hemocorrection;
- blood transfusion;
- the introduction of large doses of hormones - hydrocortisone, dexamethasone;
- antibacterial agents;
- drugs that relieve the allergic mood of the body - Suprastin, Tavegil;
- external treatment of affected organs;
- symptomatic therapy - according to indications, painkillers, restorative medications;
- diet with the exception of products with an allergic mood.
Treatment of Stevens-Johnson syndrome is aimed at preventing further progression of the disease with the exception of the effect of the allergen on the body.
Possible complications and prognosis
Late started treatment threatens with various complications from the internal organs. The course of a malignant pathological process can cause bleeding from the organs of excretion, pneumonia, and renal pathology.
In addition, the development of colitis may be a complication, and eye damage will be complicated by blindness.Given such severe complications of the underlying disease, which can be fatal in 10% of patients, early diagnosis and treatment play a very important role.
Only in this case, the prognosis for life will be positive. Late dates of initiation of drug therapy give an unfavorable prognosis, since while preserving the patient’s life, a limitation in working capacity and access to disability occur in most cases.
Prevention
To prevent this severe pathology, a number of preventive measures must be observed, such as:
- quitting smoking and drinking alcohol;
- following a diet in the diet and refusing foods that can cause allergies;
- in the presence of chronic pathology of the internal organs, medical supervision of a doctor and timely treatment of their exacerbations;
- high-quality treatment of colds seasonal viral pathologies;
- exclusion of uncontrolled medication;
- moderate physical activity in the form of swimming, fitness;
- hardening of the body.
By following these simple recommendations for disease prevention, you can avoid such a severe damage to the body. And when the first signs appear, a specialist consultation and treatment prescribed by him will allow to stop the development of the pathological process in time and prevent various complications.
