Stevens Johnson syndrome (SJS) is a rare skin disorder that can be fatal, often triggered by an allergic reaction to medications or infections. Symptoms include flu-like symptoms, rashes, skin peeling, blisters, and inflammation of mucous membranes. Treatment includes identifying the cause and stopping drug therapies or administering antibiotics in severe cases. Patients may face a recurrence and should avoid closely related drugs and inform healthcare professionals of their history with the disease.
Stevens Johnson syndrome (SJS), also called erythema multiforme major, is a rare skin disorder that has the potential to be fatal. In some cases, the cause is not known, but the most common triggers are an allergic reaction to medications or an infection.
Medications most often linked to Stevens Johnson syndrome include sulfonamides and penicillin, which are used to fight infections; anticonvulsants, which treat seizures; and nonsteroidal anti-inflammatory drugs (NSAIDs), which relieve pain and reduce inflammation. Some examples of NSAIDs are Allopurinol, Phenytoin, Carbamazepine, Vioxx, barbiturates, aspirin and ibuprofen.
The types of infections that can cause Stevens Johnson syndrome include herpes simplex or shingles, influenza, human immunodeficiency virus (HIV), diphtheria, typhoid, and hepatitis. Sometimes, SJS is the result of radiation therapy or ultraviolet light. Another form of the skin disease SJS is referred to as toxic epidermal necrolysis (TENS). Penicillin has been linked to TENS.
SJS symptoms take up to two weeks from the time the patient takes the medicine to manifest. The first warning signs occur over several days of flu-like symptoms: cough, headache, pain and fever, vomiting and diarrhea. The danger can escalate into rashes, skin peeling and skin lesions; blisters, especially around the mouth, eyes, vagina, or other areas; and inflammation of mucous membranes, which line internal organs and some exposed body parts such as the nose, lips, and ears.
Stevens-Johnson syndrome can affect people of any age group, but people most at risk are older people, possibly because they use more medications associated with SJS; and those living with acquired immune deficiency syndrome (AIDS), a condition caused by a virus and which impairs the effectiveness of the immune system. Stevens-Johnson syndrome has also occurred among children taking ibuprofen-based medications such as Advil® and Motrin® for children. Carrying a gene known as HLA-B12 can make a person more susceptible to SJS.
Isolating the cause of Stevens-Johnson syndrome and TENS, if possible, is the first step in identifying the proper treatment. When stimulated by drug therapies, prescriptions can be stopped immediately.
If caused by an infection, doctors are likely to treat Stevens-Johnson syndrome and toxic epidermal necrolysis first with an antibiotic. In severe cases where the patient loses fluid or skin, treatment should be managed in sterile environments – hospitals or burn units – to prevent progression of the infection. Some people may need to replace the liquid with an intravenous injection.
Those who have survived a first attack with Stevens-Johnson syndrome may face a recurrence. It is recommended to avoid closely related drugs if the drugs caused the first episode and to inform healthcare professionals of a history with the disease. Patients may also choose to wear a medical information bracelet or necklace.
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