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Stevens Johnson Syndrome and Related Diseases Linked to Certain Drugs

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Case ID: 3620 | Drugs / Medical | 08/17/2004

Kahn Gauthier Swick is investigating cases in which persons have contracted Stevens Johnson Syndrome (SJS), Toxic Epidermal Necrolysis Syndrome (TENS), or Erythema multiforme (EM) after taking any of a number of drugs, including prescription nonsteroidal anti-inflammatory drugs Daypro (oxaprozin), and Bextra (valdecoxib), and the anti-rheumatoid drug Arava (leflunomide). The syndromes are potentially life-threatening inflammatory skin disorders that can also cause lesions on internal organs. If you or a loved one has suffered from any of these debilitating conditions, please contact Kahn Gauthier for more information.

All three disorders are immune-complex-mediated hypersensitivity disorders (i.e. allergic in origin). SJS may cause lesions in the mouth, eyes, on the body, and on internal organs. In some cases, permanent skin damage and scarring occur. Lesions on internal organs can cause serious inflammation of the lungs, heart and kidneys. Prescription nonsteroidal anti-inflammatory drugs (NSAIDs), such as Daypro (oxaprozin), are one of the leading classes of drugs that cause the diseases. Over-the-counter NSAIDs, sulfa antibiotics, antibiotics, barbiturates, anticonvulsants, anti-tuberculosis drugs, and the drug Zyloprim (allopurinol) have also been implicated in their onset.

In Stevens Johnson Syndrome, patients have blistering of mucous membranes, typically in the mouth, eyes, and vagina, and patchy areas of rash. In TENS (sometimes referred to as Lyell's syndrome), there is a similar blistering of mucous membranes-- in addition to blistering, though, the entire epidermis peels off in sheets from large areas of the body. Both disorders can be life threatening. Erythema multiforme also occurs in response to medications, infections, or illness. Medications associated with EM include sulfonamides, penicillins, barbiturates, and phenytoin. The disorder is believed to involve damage to the blood vessels of the skin with subsequent damage to skin tissues. Approximately 90% of Erythema multiforme cases are associated with herpes simplex or Mycoplasma infections.

With more Americans taking antibiotics and other prescription drugs, these potential deadly adverse drug reactions are increasing. An under-reported and under-recognized condition, SJS is a devastating reaction affecting the skin and mucous membranes, causing severe burning, blistering and sloughing of involved tissue. Up to 27% of those affected by SJS, TENS, or EM incur long term eye damage or vision loss. As many as 15% of patients with the disease die from the condition. Because many physicians and emergency facilities are not familiar with the symptoms, treatment is frequently delayed, further exacerbating the condition.

To recognize SJS. TENS, or EM in their earliest stages, watch for the following symptoms when taking prescribed medications:

• Persistent fever;
• Burning or blistering of the mucous membranes, i.e.: eyes, ears, mouth, nose, genital area;
• Rash, blisters or red splotches on skin;
• Flu-like symptoms; and/or
• History of a reaction to prescribed drugs or over the counter medications.

In one estimate, SJS is reported to effect three to eight people per million per year in the U.S. However, the frequency could be much higher since only 1% of adverse drug reactions is reported, according to the Food and Drug Administration.

"SJS is not as rare as we are led to believe," said Jean McCawley, president of the SJS Foundation. "Since setting up the SJS Foundation and launching our website, we've been contacted by thousands of individuals. During the winter months, we learn of 15 new cases a week, and that's only people with Internet access."


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