Stevens-Johnson syndrome is a severe skin disease that can present as an allergic immunological reaction to various drugs, infections and malignant processes. Skin symptoms such as cocardia form not only on the skin but also on the mucous membrane of patients. During treatment, the primary cause of the reactions is eliminated as much as possible.
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. For example, erythema exsudativum multiforme occurs in the upper corium and corresponds to an acute dermal reaction to inflammation. It is an erythema, or reddening of the skin, in the context of infections with herpes simplex, streptococci, or paraneoplasia. In addition, drugs may be responsible for the erythema. Stevens-Johnson syndrome is a form of erythema exsudativum multiforme and accordingly belongs to the more severe allergic drug or infection reactions of the skin. In the past, the term erythema exsudativum multiforme majus was used for the allergic reaction. However, this use of the term is now obsolete, as different etiologies exist for the two skin reactions. Stevens and Johnson are considered the first describers of Stevens-Johnson syndrome and gave the symptom complex its name.
Causes
In about half of all cases, the cause of Stevens-Johnson syndrome is allergic reactions to certain drugs. 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 to date. Stevens-Johnson syndrome is not necessarily associated with drug allergy. More rare 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 a background of mycoplasma infections and other bacterial or viral infections.
Symptoms, complaints, and signs
Clinically, Stevens-Johnson syndrome manifests as severe general complaints at its acute onset. The patient’s general condition deteriorates abruptly, often culminating in high fever and rhinitis. In almost all cases, the mucous membranes are massively involved in the reaction. Erythema with blurred borders and central dark coloration forms on the skin and mucous membranes in a very short time. This characteristic symptom is also called atypical cocardia. Blisters appear in the mouth, throat and genital area. Often the skin lesions hurt or are otherwise sensitive to touch. The skin of the eye is also usually not spared from symptoms. In many cases, erosive conjunctivitis occurs. In most cases, patients can no longer open their mouths. This results in difficulties with food intake. In the course of the rather severe skin reaction, complications may occur. Lyell’s syndrome, also known as scalded skin syndrome, is considered the maximum complication.
Diagnosis and course of the disease
Although Stevens-Johnson syndrome in all cases shows a thoroughly typical symptom picture, a skin biopsy is usually performed as part of the diagnostic process. The tissue sample is used to confirm or exclude suspected diagnoses of Stevens-Johnson syndrome. Specific laboratory parameters or special tests are not available as part of the diagnostic process. However, histopathology typically shows necrotic keratinocytes. A vacuolization of the basement membrane is equally informative. Subepidermal clefting may also be suggestive of Stevens-Johnson syndrome. The prognosis is rather unfavorable for patients with the syndrome compared to most other skin reactions. The lethality rate is about six percent. If the syndrome develops into Lyell’s syndrome during its course, the lethality is as high as about 25 percent. In less severe cases, the skin symptoms heal without leaving scars. At most, skin pigment disorders remain.However, due to mucosal strictures or adhesions, the risk for complications is high.
Complications
Stevens-Johnson syndrome can be very severe. The ulcers leave scars as they heal. Mucosal shrinkage may also occur. Because the mucous membranes are very irritated during the acute phase of the disease, there is a risk of secondary infection with local pathogens, such as fungi or bacteria. Furthermore, there may be a severe loss of fluids and, as a consequence, dehydration and physical or mental deficits. If the inflammation spreads to the eye skin, this can result in conjunctivitis. A severe complication is Lyell’s syndrome, in the course of which the skin detaches and becomes necrotically scarred. In one out of four cases, the sequelae are fatal. In less severe cases, skin pigment disorders remain. Further complications may occur due to adhesions and changes in the mucosal structure. Therapy of Stevens-Johnson syndrome involves various risks – for example, preparations such as macrolide antibiotics and tetracyclines. Both agents can cause side effects and interactions, and allergic reactions occasionally occur. Intensive care measures such as infusions can lead to infections or injuries. Serious complications such as blood clots or tissue necrosis also cannot be ruled out as a result of improper treatment.
When should you see a doctor?
In Stevens-Johnson syndrome, the affected person is dependent on a visit to a physician. Since this cannot result in an independent cure and the symptoms of the syndrome usually worsen if left untreated, a doctor should be consulted at the first symptoms that indicate the syndrome. Only early detection and treatment of this disease can prevent further complications. A doctor should be consulted for Stevens-Johnson syndrome if the affected person suffers from a very severe fever. As a rule, the fever occurs abruptly and does not disappear on its own. Blisters appear in the mouth and throat, and most patients also suffer from inflammation of the conjunctiva. If these symptoms occur, the affected person must contact a doctor immediately. Stevens-Johnson syndrome is usually treated in a hospital. In emergencies or if the symptoms are very severe, an emergency doctor should be called. Whether the disease can be completely cured cannot generally be predicted.
Treatment and therapy
Various procedures can be considered for the therapy of patients with Stevens-Johnson syndrome. In all cases, the focus of therapeutic measures is to eliminate the primary cause of the reaction. The cause is usually medications given during the previous weeks. The drugs should be discontinued or replaced promptly. 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 the case of infected children. Glucocorticoids are controversial in their efficacy in the context of Stevens-Johnson syndrome and are not given because of the increased mortality rate. In particular, patients with oral and pharyngeal symptoms more readily experience respiratory tract infections in the setting of corticosteroid treatment, according to recent studies. In addition to corticosteroid administration, immunoglobulin administration is also dangerous. Extensive skin detachments are treated according to the rules of therapy 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. For local therapy, disinfectant solutions and moist compresses are indicated.
Prevention
Stevens-Johnson syndrome is an allergic immunologic reaction. The infection-associated form of the skin disease can be prevented by general infection prophylaxis. However, because the syndrome can also develop on the basis of drug-induced allergies or malignant processes, there are few fully comprehensive preventive measures available.
Follow-up
Stevens-Johnson syndrome requires comprehensive follow-up care.The skin disease can cause various complaints, which sometimes persist for some time after the cause has been treated. The dermatologist can examine the abnormal areas and prescribe appropriate medication and measures. If treated early, Stevens-Johnson syndrome should resolve quickly. After a few weeks, the doctor can be consulted again. During the follow-up, it is checked whether the prescribed medication is having its effect. If a drug has been discontinued, it is checked whether the drug was actually the cause of the symptoms. If necessary, several tests must be carried out until the desired result is achieved. Follow-up care can accordingly extend over weeks or even months. As a rule, however, dermatostomatitis can be treated well without subsequent complaints being expected. Follow-up care for Stevens-Johnson syndrome is carried out by the general practitioner. Depending on the severity of the disease, various specialists may be involved in the therapy. In addition to the dermatologist, internists may also be responsible for treatment and follow-up care. After local therapy, the treated areas must be checked for swelling or abnormalities. If complications occur, adjustment of the therapy is necessary.
What you can do yourself
Self-help is not advisable for Stevens-Johnson syndrome. Rather, patients should seek medical attention immediately. There may be a danger to life. The following self-help measures are therefore intended only as supplementary self-therapy. Since this is an allergic reaction, general infection prophylactics sometimes promise relief. However, not all possible causes can be excluded. In three quarters of all cases, drugs trigger Stevens-Johnson syndrome. Although discontinuation inhibits the typical symptoms, it can lead to other problems. Patients should therefore first consult their physician before discontinuing any medication. Rest and recuperation are the best way to recover from many diseases, including Stevens-Johnson syndrome. Doctors also advise moist compresses, which you can apply yourself without much effort. These should be provided with a camomile extract. Appropriate sitz baths for the genital area are also available in drugstores. For the mouth area there are rinses with chamomile. Rashes are characteristic of Stevens-Johnson syndrome. Ointments with a high zinc content ensure that the redness subsides. These are also free and can be purchased without a prescription.
