Stevens-Johnson Syndrome

What is Stevens-Johnson Syndrome?
Stevens-Johnson Syndrome (SJS) is a condition described by resulting severe skin reactions that include rashes and blisters that can progress to third degree burn-like intensity. SJS may result from many causes, though adverse drug reactions are often reported in conjunction with the condition. Pfizer, Inc. removed Bextra® from the market in April of 2005 for this very reason. SJS was once thought to be rare, but that may have been the result of a general lack of awareness. As recognition and understanding have increased, many have realized that SJS is not as rare as once believed. Misdiagnosis and overlooked symptoms are still prevalent, however, and only serve to exacerbate this serious - and sometimes fatal - condition.

SJS occurs almost twice as often in males as females and more often in the young than the old, though rarely in infants. However, anyone at anytime can suffer from this horrifying malady.

SJS may also be referred to by the following technical terms, which indicate levels of severity:

• Erythema multiforme minor
• Erythema multiforme major
• Toxic epidermal necrolysis (TEN)
  

What causes SJS?
SJS has been documented as a side effect of or reaction to several situations, including:

Drugs, including but not limited to:

• Non-Steroidal Inflammatory Drugs (NSAIDs), both prescription and over-the-counter
• Barbiturates
• Anti-convulsants
• Sulfa or sulfa-containing drugs
• Antibiotics
• Malignant diseases
• Viral or bacterial infections
  

Between 25% and 50% of all cases of SJS are reportedly idiopathic: cases that arise spontaneously with no apparent origin or cause.

What are the symptoms of SJS?
The symptoms of SJS often progress in severity as follows, especially if left untreated: Flu-like symptoms, including fever, fatigue, headache, sore throat, cough, nausea, etc.

• Blisters or rashes of the skin
• Blisters or lesions on the mucous membranes (thin moist tissues that line body cavities) of the mouth, nose, eyes, genitals, urinary tract, GI tract, or respiratory tract
• Ulceration of blisters leading to shedding of the skin
  

Anyone at risk for SJS, especially people exposed to the SJS causes mentioned above, should seek immediate medical attention at the first appearance of rash, blisters on the mucous membranes, or signs of hypersensitivity, including allergic reactions that involve breathing problems. Early diagnosis by a medical professional could result in a better chance of focused, effective treatment.

How is SJS treated?
The first step after diagnosis of SJS is to determine the underlying cause. If it is a drug reaction, as is often the case, the offending agent should be stopped immediately in hopes of preventing further reaction. If the cause is an infection, then the appropriate treatment should be immediately pursued in addition to the treatment of SJS symptoms. Oral and topical corticosteroids are often used to reduce the rash and swelling symptoms in less severe cases or to prevent the progression of worsening symptoms.

More severe SJS cases are often referred to various experts depending upon the body systems involved. For those with blisters involving the eyes and eyelids, examination by an ophthalmologist is recommended so precautions can be taken to avoid permanent eye damage. Those who experience severe burn-like effects of the skin are often referred to burn treatment centers, where their damaged skin and compromised body systems are treated not unlike that of a burn victim. These severe cases need fluid replacement, scrupulously sterile environments for infection prevention, and skin graft consultation that burn centers and their resident professionals can provide.

What are the long-term effects of SJS?
In severe cases, because of the deep-tissue involvement of the body’s mucous membranes, long-term and far-reaching effects of SJS are likely, if not inevitable.

• Scarring of the skin, GI tract, respiratory tract and other organ systems, and resulting complications
• Blindness or other vision problems
• Body temperature regulation problems
• In addition to these and other long-term health effects, this condition sometimes leads to death.
  

Those who have suffered a drug reaction-related case of SJS should probably avoid the offending drug and other related drugs in the future. Immediate relatives of the victim should pursue a similar course of action as these hypersensitivity issues are sometimes found to be genetically linked. Furthermore, previous sufferers of SJS should be aware that they are generally more susceptible to future recurrences of SJS.

What should I do if I have been affected by SJS?
SJS cases can be extremely complex, often resulting in overwhelming medical bills for extensive treatment and long-term effects that can be permanently disabling. Consequently, you may need an experienced SJS lawyer on your side to help meet your legal needs. At our law firm, we believe that people who have experienced SJS need an attorney who understands what they’re going through and is prepared to advocate on their behalf. If you or someone you know has suffered from this debilitating skin condition, we would like to help. For more information, visit the Stevens-Johnson Syndrome Foundation at www.sjsupport.org.

Never discontinue taking any medication without first consulting with your physician. This article is for informational purposes only. It is not intended to constitute medical advice and should not be viewed as such. For medical advice, you should always consult with your physician.

Bextra® is a registered trademark of Pfizer, Inc. and is used here only for the purpose of identifying the product in question.

This law firm is not affiliated with, sponsored by, or associated with Pfizer, Inc. or the FDA.