Stevens-Johnson Syndrome – a devastating disease

Ever since medical drugs were introduced, they were meant to be a life saver. For whatever purpose it has been used – be it to relieve pain, to treat infection, or to treat medical conditions – it has achieved its goals most of the time. However, during the course of therapy, drugs have also caused undesirable side effects. On most occasions the side effects are minor and tolerable, but on few rare occasion side effects have been severe and deadly. Stevens-Johnson Syndrome (SJS) is one such deadly drug reaction.

What makes SJS deadly is the fact that it is a physically and psychologically devastating disease. Since there is no mandatory reporting of adverse drug reactions, the incidence of SJS is not clearly known. A young woman from California who is battling for survival after using an antibiotic prescribed by her friend, and a boy who died from complications arising from SJS at Scranton, PA, are two recently reported cases of SJS in the United States.

SJS was first described in 1922. It was described as extraordinary, generalized skin eruptions. The causes of SJS are multiple and grouped in medical literature as iatrogenic (secondary to drugs), infective and idiopathic (unknown reason).

Drugs usually related to SJS include

• Antibiotics such as penicillin, sulfonamide, levofloxacin, tetracycline, erythromycin and cephalosporin
• Pain killer like non steroidal anti-inflammatory drugs including aspirin, ibuprofen, phenylbutazone
• Anticonvulsants such as phenytoin, carbamazepine
• Antigout drugs like allopurinol
• Antipsychotics

Mycoplasmal pneumonia is one common infection related to SJS. A few cases of SJS are also reported post herpes simplex infection.

Symptoms and Signs:
Drugs and infective organisms apart from their usual effects, sometimes, on rare occasions cause unwanted harmful substance to be released from blood vessels leading to swelling and skin epidermis necrosis. The affected areas get easily peeled off with minimal pressure. Those skin eruptions and peeling skin / mucous membrane are the hallmark features of SJS.  Before skin peeling starts, patients usually complain of low to high grade fever, headache, body ache, sore throat and malaise. The described prodromal symptoms occur a week or two before skin problems develop.

Besides affecting local skin, areas of mouth, tongue, eyes (conjunctiva), genitalia, gut and breathing pipe are also affected by similar mucosal peeling. Raw and exposed areas are easily prone to infection. Fluid and electrolyte loss can lead to organ failure.

Though skin eruptions heal in 2-4 weeks, in some cases, features leave behind a permanent disability and psychological damage.

Treatment:
Early recognition and treatment is key when it comes to dealing with SJS and severe drug reactions. When diagnosed, the doctor should identify possible culprits and treat it. If there is a medical drug involved, it should be stopped immediately. Other treatment options include:

• Treating the raw wound with adequate and regular sterile dressing. A relatively new product referred to as biologic dressing is preferred for rapid healing. Though skin grafting isn’t always necessary, it may be needed in some patients. Besides dressing, antiseptic is often used to prevent infection. Please make a note that silver sulfadiazine, which is an agent commonly used in treating burn wounds, should not be applied as there are reports that this agent can lower immune response.
• Steroids in high dosage are only used in certain controversial circumstances due to adverse events associated with its use.
• Oral antibiotic would be needed if infection is severe and involves systemic organs.
• Along with those, intravenous fluid and electrolyte correction and symptomatic measures will form the basic line of treatment.

Stevens-Johnson Syndrome is a deadly and devastating disease both physically and psychologically. However, early recognition and aggressive management will minimize morbidity and mortality associated with it.  Education and reassurance is extremely important. Constant support of both patient’s caretakers and nursing staff is necessary to relieve anxiety and post acute illness damages.