Stevens-Johnson syndrome is characterized by skin lesions of erythema multiforme, extensive, painful erosions with foci of tissue necrosis and epidermal shedding. What are the causes of Stevens-Johnson syndrome and how is it treated?
Stevens-Johnson syndrome(ZSJ, Latinerythema multiforme major , English Stevens-Johnson syndrome - SJS) is life-threatening an acute and rapid dermatological reaction that develops in people who are hypersensitive to various pharmacological, infectious or chemical agents.
The disease develops especially often in patients after the use of certain antibiotics, salicylate drugs and barbiturates.
Stevens-Johnson syndrome is not a common disease, it is reported in the literature that it affects an average of 2 to 5 people per million during the year. People of all ages are ill, relatively often children and adolescents.
Stevens-Johnson syndrome is slightly more often diagnosed in women than in men. The mortality rate is around 5-10%.
Stevens-Johnson syndrome: causes
The causes of Stevens-Johnson syndrome are not well known. It is supposed to be a reaction of the body's immune system with hypersensitivity to certain chemicals and their metabolites or microorganisms. It leads to massive apoptosis (that is, programmed cell death) of the cells that make up the epidermis.
Stevens-Johnson syndrome is especially often considered an undesirable effect of certain drugs, especially antibiotics (sulfonamides, tetracyclines, penicillins), antiepileptic drugs (barbiturates, carbamazepine, lamotrigine), non-steroidal anti-inflammatory drugs from the oxicam group, allopurinol ( used in the treatment of gout), agents used in the prevention of malaria and some antidepressants.
It is also worth mentioning that the Stevens-Johnson syndrome can also develop in the course of infections, especially those caused by the bacteriumMycoplasma pneumoniae , which is responsible for the development of atypical pneumonia, as well as the herpes virus normal, flu or hepatitis.
Stevens-Johnson syndrome:symptoms
Stevens-Johnson syndrome is an acute mucocutaneous reaction to various chemicals, pharmacological or infectious agents.
Among the most common and characteristic skin lesions observed in patients, there are unstable blisters and blisters filled with serous fluid, which break easily and leave painful erosions (i.e. areas devoid of epidermis) with tissue necrosis.
The lesions initially appear on the mucous membranes of the mouth, throat and genitals, on the lips, and sometimes on the mucous membranes of the eyes and nose.
With time, changes in the form of erythematous spots are also observed on the skin of the face, torso, and in rare cases, even the whole body.
Moreover, in the course of Stevens-Johnson syndrome, the creaking and atrophy of the nail plates are described.
It is the acute necrosis of the epidermis and mucous membranes, which covers less than 10% of the body surface, that makes the diagnosis of Stevens-Johnson syndrome.
In addition to the characteristic skin lesions, patients almost always have general symptoms that precede their appearance by several or several days.
These include:
- headache
- high fever
- chills
- weakness
- feeling unwell
- sore throat
- cough
- conjunctivitis
- muscle and joint pain
The disease lasts for 3-6 weeks.
Stevens-Johnson syndrome: diagnosis
The diagnosis of Stevens-Johnson syndrome is made by a doctor specializing in skin diseases based on the clinical picture, the percentage of mucosal surface and skin involvement by the disease process, as well as immunopathological examination of the skin segment.
It is worth mentioning that, according to the literature, the Stevens-Johnson syndrome and toxic epidermal necrolysis are the same disease entity that differs only in the degree of the lesion severity and the area of the skin it covers.
Eruptions covering less than 10% of the body surface allow the doctor to diagnose Stevens-Johnson syndrome, while the involvement of more than 30% of the body surface indicates the presence of toxic epidermal necrolysis.
Covering 10-30% of the skin's surface with a disease process is called the syndrome of overlapping both of the above units.
Stevens-Johnson syndrome: differentiation
Stevens-Johnson syndrome is a dermatological disease characterized by erythema multiforme eruptions, vesicles and blisters, erosions, as well as foci of necrolysis andcreeping epidermis.
The course of many skin diseases is similar, therefore, before making a final diagnosis, a doctor specializing in dermatology should take into account other conditions that cause similar symptoms, especially such as:
- hives
- staphylococcal exfoliative dermatitis
- herpes infection
- common pemphigus
- pemfigoid
- foot-and-mouth disease
- Kawasaki disease
Stevens-Johnson syndrome: treatment
Treatment of Stevens-Johnson syndrome should take place in a hospital, under the supervision of dermatology specialists and personnel experienced in the treatment of burns. It depends mainly on the etiological factor and the severity of erythema multiforme.
Therapy is about identifying and discontinuing medications or other factors that are responsible for triggering a skin reaction.
Pharmacological treatment is based on the topical application of ointments and creams containing glucocorticosteroids (the value of systemic corticosteroid therapy, however, is debatable).
Your doctor may also decide to use cyclosporin A, broad-spectrum antibiotics or antihistamines.
It is worth remembering to include means and dressings intended for the care of burn wounds, which support the healing process, as well as analgesic treatment, which significantly affects the comfort and well-being of the sick person.
Stevens-Johnson syndrome: complications
The greatest number of complications in people suffering from Stevens-Johnson syndrome is associated with the development of infections and secondary superinfections of skin lesions.
Depending on the extent of the skin surface covered by the eruptions, thermoregulation disorders and water and electrolyte imbalances may also occur. The presence of eruptions within the eye can lead to conjunctival overgrowth and even corneal damage, which may result in serious vision problems.
In addition, there is a high risk of urinary, digestive and respiratory complications, and the likelihood of relapse.
Mortality in Stevens-Johnson syndrome is around 5-10%, while in toxic epidermal necrolysis it is as high as 25-30%.