Often a consequence of certain types of medications, Stevens-Johnson syndrome can sometimes be caused by infections or other factors.
Skin peeling is one of the characteristic symptoms of the syndrome in question; Patients undergoing its development are usually hospitalized and undergo the necessary supportive care and treatment. If the syndrome is caused by drugs, their intake should be stopped immediately.
If diagnosed promptly, the prognosis is generally good.
and mucous membranes (necrolysis) whose consequences can be extremely serious.
The annual incidence of Stevens-Johnson syndrome is approximately 1-5 / 1,000,000, therefore fortunately this is a very rare reaction.
Several sources consider Stevens-Johnson syndrome as a limited variant of toxic epidermal necrolysis (or Lyell's syndrome, a particular type of polymorphic erythema), characterized by a similar but more extensive and severe symptomatology than that of Stevens-Johnson syndrome.
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: what are the differences?
The symptom pictures of Stevens-Johnson syndrome and toxic epidermal necrolysis are very similar to each other. Both are characterized by the destruction and subsequent desquamation of the skin which, however, in the syndrome object of the article affects limited body areas (less than 10% of the entire body surface); while in toxic epidermal necrolysis it affects larger areas of skin (over 30% of the body surface).
The involvement of 15% to 30% of the skin surface is, on the other hand, considered as an overlap between Stevens-Johnson syndrome and toxic epidermal necrolysis.
(such as, for example, co-trimoxazole and sulfasalazine);Other Causes
Other possible causes responsible for the onset of Stevens-Johnson syndrome are:
- Infections of a viral or bacterial nature (in particular, sustained by Mycoplasma pneumoniae);
- The administration of some types of vaccines;
- Graft versus Host Disease (or GVHD, from English Graft-Versus-Host Disease).
What people are at risk for developing Stevens-Johnson Syndrome?
The risk of developing Stevens-Johnson syndrome is greater:
- In HIV positive and AIDS patients;
- In patients with impaired immune system (for example, due to drugs);
- In patients with infections caused by Pneumocystis jirovecii;
- In patients with systemic lupus erythematosus;
- In patients suffering from chronic rheumatic diseases;
- In patients with a previous history of Stevens-Johnson syndrome.
Finally, the hypothesis of the existence of a certain genetic predisposition to the development of Stevens-Johnson syndrome has also been advanced.
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At this stage, many patients may also experience an unexplained burning sensation and / or pain in the skin.
In case Stevens-Johnson syndrome is caused by medication, the above prodromal symptoms appear within 1-3 weeks after starting therapy. Skin and mucosal symptoms below described, however, appear after 4-6 weeks from the beginning of the same.
Symptoms affecting the skin and mucous membranes
After the onset of the aforementioned prodromal symptoms, symptoms affecting the skin and mucous membranes arise. They begin with a "flat and red rash that generally begins on the face, neck and trunk and then spreads to the rest of the body. In the specific case of Stevens-Johnson syndrome, this rash affects less than 10% of the surface. corporeal.
The appearance of the rash is followed by the formation of blisters that tend to exfoliate within 1-3 days. Blisters can also appear in the genitals, hands, feet; they occur on the mucous membranes (for example, in the mouth, throat, etc.) and may even involve the internal epithelia, such as those of the airways, urinary tract, etc. The eyes are also generally affected by the formation of blisters and crusts: they are swollen, red and painful.
In addition to marked skin peeling, nail and hair loss can also be encountered.
Clearly, in such a condition, the patient perceives considerable pain which is associated with an "equally noticeable swelling. Furthermore, depending on the areas where blisters form and where desquamation occurs, the patient may develop breathing difficulties, difficulty in urination, difficulty keeping your eyes open, difficulty swallowing, speaking, eating and even drinking.
Complications of Stevens-Johnson Syndrome
Complications of Stevens-Johnson syndrome occur mainly due to necrosis and subsequent peeling of the skin and mucous membranes. Lacking the barrier function typically exercised by the skin, in fact, we can encounter:
- Massive loss of electrolytes and fluids;
- Contraction of various types of infections (bacterial, viral, fungal, etc.) which can also lead to sepsis.
Another serious complication consists in the "onset of" insufficiency affecting various organs (multiorgan insufficiency).
skin resulting in histological examination, although this is not a frequently performed procedure.
Unfortunately, early diagnosis, when the syndrome is still in its infancy and manifests itself with prodromal symptoms, is not always possible. In fact, since these symptoms are rather nonspecific, it could lead to an "incorrect evaluation, with consequent delay in" identifying the real cause of the patient's malaise.
What diseases shouldn't Stevens-Johnson Syndrome be confused with?
The manifestations and symptoms induced by Stevens-Johnson syndrome may be similar to those induced by other diseases involving the skin and mucous membranes, with which, however, it should not be confused. More in detail, the differential diagnosis must be placed in relation to:
- Dell "polymorphic erythema minor and major;
- Of toxic epidermal necrolysis or Lyell's syndrome, if you prefer;
- Toxic shock syndrome;
- Of exfoliative dermatitis;
- Pemphigus.
If the syndrome is caused by taking drugs, treatment with the drugs should be stopped immediately.
Unfortunately, although there are some drugs that can be administered to try to stop the progression of the syndrome, there is no real cure for Stevens-Johnson syndrome. In any case, patients hospitalized because of the syndrome in question must receive adequate supportive treatments.
Supportive Therapy
Supportive care is essential to ensure patient survival. It may vary according to the conditions of the latter.
- Lost fluids and electrolytes will need to be administered parenterally. The same applies to nutrition if the patient is unable to provide for it autonomously due to the lesions reported on the skin and mucous membranes of the mouth, throat, etc.
- Patients with eye involvement will need to undergo specialist visits and undergo targeted treatments to prevent, or at least limit, the damage induced by the syndrome.
- Skin lesions typical of Stevens-Johnson syndrome need to be treated daily and treated in the same way as burns.
- In the presence of secondary infections, it is necessary to proceed with the treatment of the latter, establishing suitable therapies against the triggering pathogen (using, for example, antibiotic drugs - provided they are not known to induce the syndrome in question - antifungal drugs, etc.) .
Drugs to Reduce the Duration of Stevens-Johnson Syndrome
In an attempt to stop Stevens-Johnson syndrome or in any case reduce its duration, it is possible to resort to the use of drugs, such as:
- Ciclosporin, given to inhibit the action of T cells.
- Corticosteroids for systemic use, in order to dampen the action of the immune system, but at the same time useful for counteracting pain.
- High-dose IV immunoglobulins (IgEV); administered early. They should arrest the activity of the antibodies and block the action of the FAS receptor ligand.
However, the use of these medicines is the subject of differing opinions among doctors.
The use of corticosteroids, in fact, is correlated with an increase in mortality and can favor the onset of infections or mask a possible sepsis; although such drugs have shown some efficacy in improving eye lesions.
Similarly, although the administration of IgEV allows to obtain good initial results, according to clinical trials and retrospective analyzes, the final results obtained are conflicting. In fact, from these experiments and analyzes, it emerged that IgEV can be not only ineffective but potentially connected to an increase in mortality.
Other Treatments
In some cases, plasmapheresis can also be performed, in order to remove residues of reactive metabolites of drugs and antibodies that could be the triggering cause of Stevens-Johnson syndrome.
In any case, the most suitable treatment for each patient will be determined by the doctor based on the condition of the same and on the basis of the stage in which the Stevens-Johnson syndrome is at when it is diagnosed.
