Stevens Johnson Syndrome (SJS) is an adverse drug reaction (ADR) and was discovered in 1922 by two pediatricians, A.M. Stevens and S.C. Johnson. While typically an allergic reaction to dugs, it can also be caused as a result of certain bacterial and viral infections. SJS is estimated to range from 2.6-7.1 cases per million per year. Although quite rare, the disease’s clinical manifestations can lead to severe adverse effects on the body and can be fatal. SJS most often occurs in young children or the elderly and seems to affect both sexes equally.
The most common causes of Stevens Johnson Syndrome are pain medications and antibiotics such as: Ibuprofen (in Advil and Motrin), Aceteminophen (Tylenol), Eiprofloxacin (Cipro), Valdecoxib (Bextra), Oxaprozin (Daypro), Celecoxib (Celebrex), Rofecoxib (Vioxx), Leflunomide (Arava), Piroxicam (Feldene), Cephalosporins, Anticonvulsants, Barbiturates, and many others. As it can be caused by such a vast amount of sources, it is often difficult to determine the causative agent. Obviously, victims should consider all drug related products recently ingested in the days and even weeks prior to the manifestation of SJS symptoms. Additionally, SJS can be caused by viral and bacterial infections including Mycoplasma or Streptococcal, genetic predisposition to autoimmune diseases, or malignancy.
SJS is characterized by a number of symptoms, which typically begin to manifest in the first few weeks of drug or medication ingestion. Among the most common are flu like symptoms including fever, soar throat, chills, and headaches. Skin lesions and red/purple spots usually follow shortly after and can develop into fluid-filled sacs/blisters. In the worst or most developed conditions, the skin seems to literally peel off the body as the blisters rupture and develop into dead skin and scar tissue. Infection can become a major problem if not treated properly. As it spreads over the skin, SJS will almost resemble a full body rash. The lesions can be very prominent around the anal/genital region and can especially cause problems around the eyes and ocular cavity. As a result of swelling and ulcerations of the cornea, vision can become permanently impaired in up to 27% of cases, possibly leading to blindness. SJS is fatal in up to 15% of untreated cases and as much as 3% of treated cases.
SJS is generally discovered through previously mentioned clinical manifestations. Immunfluorescence studies and/or skin biopsies may be performed to make or confirm diagnosis.
Patients should discontinue use of the drug or offending medication immediately and continued care of developed skin conditions is necessary to prevent infection or furtherance of the disease. Routine eye care using antibiotics and antiseptic eye drops should be used and oral and nasal debris should be removed. The mouth should be treated and sprayed with antiseptic agents as well. The most effective treatment seems to essentially consist of preventing or mitigating further development of the disease. There does not seem to be any real way to prevent SJS other than to refrain from medication ingestion.
The most relevant legal issues involve the drug Bextra. Bextra is typically proscribed for the elderly to relieve arthritis pain. Manufactured by Pfizer, Bextra was more or less an alternative to Vioxx and is a COX-2 selective non-steroidal anti-inflammatory drug. Thus, it is able to selectively block the COX-2 enzymes responsible for arthritis inflammation while not disturbing the COX-1 enzymes that protect the stomach. Recently there has been an abundance of legal advertisement associating Bextra to SJS cases and heart attacks. Claims tend to involve inadequate warning and off-label promotion. Current controversy also exists regarding FDA approval despite only six months of clinical trials. In November of 2002, the FDA issued a warning regarding Bextra and its numerous side effects basically resulting in Pfizer pulling it from the shelves. Legal issues regarding Bextra and SJS seems to still be in the very early stages of litigation.