Description of Stevens-Johnson Syndrome Erythema multiforme (EM) is acute inflammatory skin disease due to multiple causes. Erythema multiforme is divided into minor and major types based on the clinical findings. Approximately 90 percent of erythema multiforme minor follow outbreaks of herpes simplex. Erythema multiforme major (Stevens-Johnson syndrome) is marked by involvement of two or more mucosal surfaces (typically oral and conjunctival [eye]).
Causes and Risk Factors of Stevens-Johnson Syndrome The syndrome has been associated with a variety of infectious and pharmacologic agents. It is most often caused by drugs, especially sulfas, non-steroidal anti-inflammatory drugs (NSAIDs), and anticonvulsants such as phenytoin. Any drug is a potential cause. Coxsackievirus, echovirus, and most commonly, herpes simplex viruses as well as mycoplasma have precipitated the syndrome. Vaccines, such as those for tuberculosis and polio, have also been implicated. In approximately 50 percent of cases no cause can be identified. Stevens-Johnson syndrome can occur in all age groups.
Symptoms of Stevens-Johnson Syndrome Typically, the mucous membranes of the oral cavity, nostril, eyes, and both the anal and genital regions. It may or may not be associated with skin lesions elsewhere on the body. Oropharyngeal (mouth) lesions may be so intolerable as to prevent eating, and there may be recurring oral ulcers. Often, the skin lesions may look like a target-lesion (3 concentric zones of color change) or as bullae (bubble-like). A painful conjunctivitis may occur in the eye, frequently with a pus discharge, and can lead to corneal scarring and loss of vision. In addition to the mucous membrane lesions, fever and exhaustion are usual. Approximately one-third of patients have pulmonary involvement with cough and patchy infiltrates (material that has permeated into the tissues) on chest x-ray. In fatal cases, renal failure and pneumonia may occur.
Diagnosis of Stevens - Johnson syndrome The diagnosis of is usually based on the clinical appearance and distribution of the skin and mucosal lesions. A skin biopsy may be performed.
Treatment of Stevens - Johnson syndrome Every attempt should be made to identify a precipitating agent, and to remove it if possible. Antibiotics are appropriate if super infection (a fresh infection added to one of the same nature already present) is suspected, or if bacterial disease, such as mycoplasma, is suspected to be the cause. Intensive supportive care is important in severe cases, including specialized care in a burn unit. Fluid replacement is often required, and meticulous oral hygiene is necessary to prevent super infection. Examination by an ophthalmologist is recommended for patients with eye lesions so that precautions can be taken to avoid permanent eye damage. Oral and topical corticosteroids are often used.
Questions To Ask Your Doctor about Stevens - Johnson syndrome
What is the cause?
What treatment do you recommend to relieve symptoms?
Will you be prescribing any medications?
What are the side effects?
What measure can be taken to avoid permanent eye damage?
Has any permanent damage occurred?
Can this occur again?
Adverse drug reactions are all too common. Most are unreported; however, they contribute to over 150,000 deaths per year, making them a leading cause of death in the U.S. The Food and Drug Administration (FDA) Commissioner has estimated that anywhere between 3-11% of hospitalizations every year are due to adverse drug reactions. People taking various prescriptions and over the counter medications are often unknowingly making themselves susceptible to serious adverse side effects that can lead to death.
Two of the most common inflammatory skin diseases due to poor reactions to over the counter and prescription drugs are Stevens-Johnson Syndrome (SJS) and a form of SJS known as Toxic Epidural Necrolysis Syndrome (TEN). Many different drugs can trigger SJS, but anti-convulsants, antibiotics and anti-inflammatory drugs are most consistently responsible for causing SJS. While anyone can develop SJS, a Caucasian predominance has been reported. It is most commonly seen in children and in women (a 2:1 ratio compared to men), if untreated, will result in death. It is possible for those who are diagnosed with SJS to die and anywhere from 3 to 15 percent of victims have suffered this consequence. Additionally, over half the people who develop SJS experience serious upper respiratory tract infections.
What is known as Stevens-Johnson Syndrome, which was discovered in 1922, is a painful skin disease marked by ulcerated lesions found on the skin of a patient. According to the Stevens - Johnson Syndrome Foundation, the symptoms of SJS include:
. Rash, blisters or red splotches on the skin.
. Persistent fever.
. The body can form blisters and lesions anywhere from the genital area to the mouth, nose, eyes and even ears.
. Swelling of eyelids and/or red eyes.
. Conjunctivitis.
. Flu-like symptoms.
Patients with these symptoms have a recent history of having taken a prescription or over the counter medication.
Various prescription drugs have been found to be strongly associated with SJS, and some have even been recalled by the FDA. Non-steroidal anti-inflammatory drugs (NSAIDs) have recently come under a great deal of scrutiny relative to causing SJS. One such drug is Bextra, an anti-inflammatory drug prescribed to treat the pain associated with osteo-arthritis and rheumatoid arthritis. The packaging of these drugs now includes strong warnings and precautions. Most are now required to have a "black box" warning, the highest warning level the FDA issues on approved drugs. The FDA has recalled or requested the manufacturer voluntarily remove these NSAIDs from the market.
Although prevention of SJS is impossible, treatments do exist to ease the painful disease. It is of the utmost necessity to stop taking the drug associated with the negative side effects/reactions. After that, hospitalization is usually necessary, combined with a variety of treatment options including care from burn units, the ICU and dermatological teams. Individuals suffering from SJS who receive prompt and thorough treatment will likely successfully recover from the illness.
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