August 2007 has been designated as Stevens Johnson Syndrome Awareness Month and has been recognized in the following states so far: Colorado, Arizona, Alabama, Connecticut and Tennessee. Stevens Johnson Syndrome (SJS) affects more than two million Americans and causes more than 140,000 deaths annually. Hopefully, more states will take notice of this deadly disease and recognize SJS Awareness month, also.
SJS is an allergic reaction that is commonly caused by drugs, many of which are over-the-counter. Allergic drug reactions are the fourth leading cause of death in the United States, and almost any drug can cause SJS, even ibuprofen. Other drugs that have been linked to SJS are Bextra, Celebrex, Daypro Allopurinol, Carbamazepine, Phenytoin and sulfa and penicillin antibiotics. SJS can also occur in response to infections or illnesses, and 25-50% of all cases derive from unknown origin.
SJS patients suffer from inflammation of the skin and mucous membranes. The disease process for SJS typically begins with a nonspecific upper respiratory tract infection. Early SJS symptoms occur in the first 1-14 day period during which fever, sore throat, chills, headaches and malaise may be present. Vomiting and diarrhea are sometimes also noted in the early symptoms. Skin and mucous membrane lesions can develop abruptly with clusters of outbreaks lasting from 2-4 weeks. Continued fever and localized worsening of the lesions suggest a superimposed infections; however, continued fever has been shown to occur in 85% of SJS cases. In some cases, the lesions of the mouth and mucous membranes are so severe that patients may not even be able to eat or drink.
Up to 15% of serious SJS patients die. In the most severe cases, the lesions lead to significant scarring of the involved organs and loss of function of the organ systems. SJS treatment is focused on the management of disease symptoms because at the current time there is no treatment able to stop the spread of the disease.
Most Stevens Johnson Syndrome patients are older than 40, but cases have been reported for children as young as three months old. The ratio of male to female occurrence is two to one. In the United States, cases of SJS tend to cluster in the early spring and winter, with a rate of about two to three cases per million persons. The incidence and frequency outside the United States is similar.