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Stevens-Johnson syndrome is a heterogeneous skin lesion that manifests itself in an acute and rapid course. Changes in the form of erythema and erosions lead to tissue necrosis. Illness is a life-threatening condition. Find out what are the symptoms, causes of Stevens-Johnson syndrome and how to treat them.
Stevens-Johnson syndrome – characteristics
Stevens-Johnson syndrome (SJS) is a serious cutaneous and mucosal reaction to the action of pharmacological, chemical and microbial agents. Skin lesions take the form of erythema multiforme, extensive, painful erosions with tissue necrosis, and are characterized by epidermis creeping. It is a life-threatening reaction that is severe and violent. Typically, SJS suffers from patients who are hypersensitive to various external factors. The disease is noted especially after antibiotic therapy, as well as after drugs from the group of salicylates and barbiturates.
Stevens-Johnson syndrome is a rare disorder that affects 2 to 5 out of a million people each year. Both children and adults suffer from SJS. Research shows that children, adolescents and women are more prone to the disease. Mortality is around 5-10%.
Stevens-Johnson syndrome – causes
Modern medicine has not fully understood the causes of Stevens-Johnson syndrome. The most common cause is hypersensitivity of the immune system to certain chemical compounds and their metabolites, and to microorganisms. In response to the above-mentioned factors, the immune system causes the mass death of epidermal cells (the so-called apoptosis).
Stevens-Johnson syndrome occurs as a side effect of certain medications:
- antibiotics (sulfonamides, tetracyclines, penicillins);
- antiepileptic drugs (barbiturates, carbamazepine, lamotrigine), oxicam non-steroidal anti-inflammatory drugs, allopurinol (a medicine used to treat gout);
- agents for the prevention of malaria;
- antidepressants.
Stevens-Johnson syndrome also occurs in the course of infections caused by:
- the bacterium Mycoplasma pneumoniae;
- herpes simplex virus;
- influenza virus;
- hepatitis virus.
Stevens-Johnson syndrome – symptoms
SJS is characterized by skin lesions that resemble blisters filled with serous fluid. The blisters are unstable, they break easily, turning into painful erosions devoid of the epidermis and with tissue necrosis. At the beginning of the disease, the changes affect the mucous membranes of the mouth, throat, mucosa of the eyes, nose, mouth and genitals. The lesions then spread over the skin of the face, torso, and sometimes even the entire body. The changes appear in the form of erythematous spots on the body. A characteristic feature of Stevens-Johnson syndrome is the shedding of the nail plates, including their disappearance.
The appearance of skin lesions is preceded by general symptoms:
- Headache;
- high fever;
- sore throat;
- cough;
- conjunctivitis;
- bad mood;
- chills;
- weakness;
- pain in muscles and joints.
The disease lasts for about 3-6 weeks. First, general symptoms appear, and a few dozen days after them, a rash appears.
Stevens-Johnson syndrome – diagnosis
The doctor diagnoses the Stevens-Johnson syndrome based on the area of acute necrosis of the epidermis and mucous membranes. If it covers less than 10% of the body surface, it indicates SJS. On the other hand, if more than 30% of the body surface is affected by the disease process, toxic epidermal necrolysis is said to occur. Toxic epidermal necrolysis and SJS are the same entity, except that toxic epidermal necrolysis covers a larger area of the body.
Stevens-Johnson syndrome – diagnosing
The skin symptoms of Stevens-Johnson syndrome are similar to many other diseases that also involve skin lesions. Therefore, it is important to exclude other disease entities.
Other diseases have similar symptoms:
- pemphigus common,
- hives
- herpes infection,
- staphylococcal exfoliative dermatitis,
- pemfigoid,
- foot-and-mouth disease,
- choroba Kawasaki.
Stevens-Johnson syndrome – treatment
It is important that Stevens-Johnson syndrome is treated in hospital under the supervision of qualified dermatologists. Therapy is based on the elimination of factors that cause skin reactions and the use of drugs: glucocorticosteroids, cyclosporin A, antihistamines, broad-spectrum antibiotics, as well as painkillers and soothing drugs. In addition to pharmacology, hygiene of wounds and erosions is introduced (gentle washing and securing wounds with dressings).
Stevens-Johnson syndrome – complications
Complications of SJS involve superinfection and infection of open skin lesions. Therefore, special care and attention should be paid to skin changes.
The patient may also experience various disorders related to the occurrence of skin lesions on the body and mucous membranes:
thermoregulation disorders;
water and electrolyte imbalance;
conjunctival overgrowth, including damage to the cornea;
complications from the urinary, digestive and respiratory systems.
About 5- 10% of patients die of Stevens-Johnson syndrome. Therefore, it is a disease that must be treated absolutely.