Stevens-Johnson Syndrome

Stevens-Johnson Syndrome

Stevens-Johnson Syndrome is a severe skin disease that can occur as an allergic-immunological reaction to various drugs, infections and malignant processes. Skin symptoms such as cockades are formed not only on the skin, but also on the mucous membrane of patients. During treatment, the primary cause of the reactions is largely eliminated.

What is Stevens-Johnson Syndrome?

Skin diseases can be congenital or acquired. One form of acquired skin disease is skin lesions in response to infections, drugs, or other toxins. Erythema multiforme, for example, occurs in the upper corium and corresponds to an acute dermal reaction to inflammation. It is an erythema, i.e. a reddening of the skin in the context of infections with herpes simplex, streptococci or paraneoplasia. For what is heart disease used for, please visit fun-wiki.com.

In addition, drugs may be responsible for the erythema. The Stevens-Johnson syndrome is a form of erythema multiforme and is therefore one of the more serious allergic drug or infection reactions of the skin. In the past, the term erythema exsudativum multiforme majus was used for the allergic reaction.

In the meantime, however, this use of the term has become obsolete, as there are different etiologies for the two skin reactions. Stevens and Johnson are considered the first to describe Stevens-Johnson syndrome and have given their name to the symptom complex.

Causes

In about half of all cases, the cause of Stevens-Johnson syndrome is an allergic reaction to certain medications. In most cases, sulfonamides, codeine, or hydantoins are responsible for the dermal reaction.

In addition, a causal relationship has been established between Stevens-Johnson syndrome and NSAIDs, NNRTIs, allopurinol, moxifloxacin, and strontium ranelate. The allergic reaction is an immunologically induced, T-cell-mediated necrosis of keratinocytes. However, the exact pathogenesis remains unclear.

Stevens-Johnson syndrome does not necessarily have to be related to drug allergies. Rarer but theoretically conceivable triggers of the immune reaction are malignant processes such as lymphomas. In addition, cases of Stevens-Johnson syndrome have been reported against the background of mycoplasma infections and other bacterial or viral infections.

Symptoms, Ailments & Signs

Clinically, Stevens-Johnson syndrome manifests itself in the acute onset in severe general symptoms. The general condition of the patients suddenly deteriorates and often leads to high fever and rhinitis. In almost all cases, the mucous membranes are massively involved in the reaction.

Erythema with indistinct borders and a dark central color develops on the skin and mucous membrane in a very short time. This characteristic symptom is also referred to as atypical cockades. Blisters form in the mouth, throat and genitals. The skin changes are often painful or otherwise sensitive to touch. The skin around the eyes is also usually not spared from symptoms.

In many cases, erosive conjunctivitis occurs. In most cases, patients can no longer open their mouths. This causes difficulties in eating. Complications can occur during the course of the rather severe skin reaction. The maximum complication is Lyell’s syndrome, also known as scalded skin syndrome.

Diagnosis & course of disease

Although the Stevens-Johnson syndrome usually shows a typical symptom picture, a skin biopsy is usually carried out as part of the diagnosis. The tissue sample serves to secure or rule out suspected diagnoses of Stevens-Johnson syndrome. Specific laboratory parameters or special tests are not available as part of the diagnosis.

However, histopathology typically shows necrotic keratinocytes. Equally meaningful is a vacuolation of the basement membrane. Subepidermal clefts can also indicate Stevens-Johnson syndrome. The prognosis for patients with the syndrome is poor compared to most other skin reactions.

The mortality rate is about six percent. If the syndrome develops into Lyell’s syndrome in the course of the disease, the mortality rate is even around 25 percent. In less severe cases, the skin symptoms heal without leaving scars. At best, skin pigment disorders remain. However, due to mucosal strictures or adhesions, the risk of complications is high.

Complications

Stevens-Johnson syndrome can be very severe. The ulcers leave scars as they heal. Shrinkage of the mucous membranes can also occur. Since the mucous membranes are very irritated in the acute phase of the disease, there is a risk of a secondary infection with local pathogens such as fungi or bacteria.

Furthermore, there can be a severe loss of fluids and, as a result, dehydration and physical or mental deficits. If the inflammation spreads to the skin of the eye, this can result in conjunctivitis. A serious complication is Lyell’s syndrome, in the course of which the skin peels off and becomes necrotically scarred.

In one out of four cases, the subsequent symptom ends in death. In less severe cases, skin pigment disorders remain. Further complications can arise from adhesions and changes in the mucosal structure. The therapy of Stevens-Johnson syndrome involves various risks – for example with preparations such as macrolide antibiotics and tetracyclines.

Both agents can cause side effects and interactions, occasionally allergic reactions occur. Intensive care measures such as infusions can lead to infections or injuries. Serious complications such as blood clots or tissue necrosis cannot be ruled out as a result of improper treatment.

When should you go to the doctor?

With Stevens-Johnson syndrome, the person affected needs to see a doctor. Since self-healing is not possible and the symptoms of the syndrome usually worsen if left untreated, a doctor should be consulted as soon as the first symptoms point to the syndrome.

Early detection and treatment of this disease is the only way to prevent further complications. A doctor should be consulted for Stevens-Johnson syndrome if the person affected is suffering from a very strong fever. The fever usually comes on suddenly and does not go away on its own. Blisters develop in the mouth and throat, and most patients also suffer from inflammation of the conjunctiva. If these symptoms occur, the person concerned must contact a doctor immediately.

Stevens-Johnson syndrome is usually treated in a hospital. In emergencies or if the symptoms are very severe, an ambulance should be called. Whether the disease can be completely cured cannot generally be predicted.

Treatment & Therapy

Various methods can be used to treat patients with Stevens-Johnson syndrome. In all cases, the therapeutic measures focus on eliminating the primary cause of the reaction. The cause usually lies in medication that has been given during the last few weeks. The medication should be discontinued or replaced as soon as possible.

Mycoplasma infections must also be considered in causal relation to the syndrome. Such infections are treated with tetracyclines or macrolide antibiotics. Antibiotics are preferable in case of infected children. Glucocorticoids are controversial in their effectiveness in the context of Stevens-Johnson syndrome and are not given due to the increased mortality rate.

According to current studies, patients with mouth and throat symptoms in particular suffer more easily from infections of the respiratory tract as part of corticosteroid treatment. In addition to corticoid administration, immunoglobulin administration is also dangerous. Large areas of skin detachment are treated according to the therapy rules for burn patients.

The most important steps in this context include intensive medical measures for fluid balance, protein balance and electrolyte balance. In addition, permanent cardiovascular monitoring is indicated for severe cases. The same applies to infection prophylaxis. If the patient’s food intake is disturbed because of the lesions, parenteral nutrition is given. Disinfectant solutions and moist compresses are indicated for local therapy.

Prevention

Stevens-Johnson syndrome is an allergic immunological reaction. The infection-associated form of the skin disease can be prevented by general infection prophylaxis. However, since the syndrome can also develop on the basis of drug allergies or malignant processes, there are hardly any comprehensive preventive measures available.

Aftercare

Stevens-Johnson syndrome requires extensive follow-up care. The skin disease can cause various symptoms, which sometimes persist for some time after the cause has been treated. The dermatologist can examine the conspicuous areas and prescribe appropriate medication and measures.

With early treatment, Stevens-Johnson syndrome should subside quickly. After a few weeks, the doctor can be consulted again. As part of the follow-up care, it is checked whether the prescribed medication is having an effect. If a drug has been discontinued, a check is carried out to determine whether the drug was actually the cause of the symptoms.

It may be necessary to carry out several tests to achieve the desired result. Follow-up care can therefore extend over weeks or even months. As a rule, however, dermatostomatitis can be treated well without any complications to be expected. Follow-up care for Stevens-Johnson syndrome is carried out by the general practitioner.

Depending on the severity of the disease, different specialists can be involved in the therapy. In addition to the dermatologist, internists may also be responsible for treatment and aftercare. After the local therapy, the treated areas must be checked for swelling or abnormalities. If complications occur, an adjustment of the therapy is necessary.

You can do that yourself

Self-help is not advisable for Stevens-Johnson syndrome. Rather, patients should see a doctor immediately. There may be a risk of death. The following self-help measures are therefore only intended as supplementary self-therapy.

Since it is an allergic reaction, general infection prophylaxis sometimes promise relief. However, not all possible causes can be ruled out in this way. Drugs cause Stevens-Johnson syndrome in three quarters of all cases. Discontinuation inhibits the typical symptoms, but can lead to other problems. Affected people should therefore first consult their doctor before giving up medication.

Rest and relaxation are the best way to recovery for many illnesses, including Stevens-Johnson syndrome. Doctors also recommend moist compresses that you can put on yourself without much effort. These should be provided with a chamomile extract. Appropriate sitz baths for the genital area are also available in drugstores. There are rinses with chamomile for the mouth area. Rashes are characteristic of Stevens-Johnson syndrome. Ointments with a high zinc content ensure that the redness subsides. These can also be purchased free of charge and without a prescription.

Stevens-Johnson Syndrome