Stevens-Johnson Syndrome: Symptoms, Causes & Treatment
Author: Thomas C. Weiss : Contact: Disabled World
Synopsis and Key Points:
Information regarding Stevens-Johnson syndrome, a disorder of the skin and mucous membranes - usually a reaction to medication or an infection.
Stevens-Johnson syndrome is a serious disorder of the skin and mucous membranes. It is usually a reaction to a medication or an infection. Often times, Stevens-Johnson syndrome starts with flu-like symptoms, followed by a red or purplish painful rash that blisters and spreads. The top layer of the affected skin dies and then sheds.
Stevens-Johnson syndrome, a form of toxic epidermal necrolysis, is a life-threatening skin condition, in which cell death causes the epidermis to separate from the dermis. The syndrome is thought to be a hypersensitivity complex that affects the skin and the mucous membranes. The most well-known causes are certain medications, but it can also be due to infections, or more rarely, cancers.
Stevens-Johnson syndrome is a medical emergency that commonly requires an affected person to be hospitalized. Treatment concentrates on elimination of the underlying cause, controlling the person's symptoms, as well as minimizing complications. Recovery following Stevens-Johnson syndrome may take weeks to months, depending upon the severity of the condition. If it was caused by a type of medication, the person will need to permanently avoid the medication and others related to it.
Symptoms of Stevens-Johnson Syndrome
People with Stevens-Johnson syndrome may experience a number of symptoms, to include hives, facial swelling, tongue swelling and skin pain. They may experience a red or purplish rash that spreads within hours to days, blisters on their skin and the mucous membranes of their nose, mouth, genitals and eyes, followed by shedding of their skin. A number of days before the rash develops, a person with Stevens-Johnson syndrome may also experience:
- Burning eyes
- Sore throat and mouth
Immediate medical attention is required for people with Stevens-Johnson syndrome. It is important for a person with this syndrome to pursue emergency medical attention if they experience any of the following signs or symptoms:
- Facial swelling
- Tongue swelling
- Shedding of skin
- Widespread, unexplained skin pain
- A red or purplish skin rash that spreads
- Blisters on the skin or mucous membranes
Causes of Stevens-Johnson Syndrome
Stevens-Johnson syndrome is rare and people have unpredictable reactions. A doctor might not be able to identify its exact cause, although usually it is triggered by an infection or a type of medication. Drugs that may cause Stevens-Johnson syndrome include the following:
- Radiation therapy
- Anti-gout medications
- Medications to fight infections such as penicillin
- Medications to treat seizures or forms of mental illness
- Pain relievers such as acetaminophen, naproxen sodium, or ibuprofen
Infections that may cause Stevens-Johnson syndrome include HIV, Hepatitis, Pneumonia and Herpes.
Risk Factors for Stevens-Johnson Syndrome
A number of factors increase a person's risk of developing Stevens-Johnson syndrome. These factors include the following:
Viral Infections: A person's risk of Stevens-Johnson syndrome might be increased if they have an infection caused by a virus such as viral pneumonia, herpes, or hepatitis.
A History of Stevens-Johnson Syndrome: If a person has had a medication-related form of the condition, they are at increased risk of a recurrence if they use the medication again.
A Family History of Stevens-Johnson Syndrome: If an immediate family member has had Stevens-Johnson syndrome, or a related condition called, 'toxic epidermal necrolysis,' a person might be more susceptible to developing the syndrome as well.
A Weakened Immune System: If a person has a weakened immune system, they may have an increased risk of Stevens-Johnson syndrome. A person's immune system can be affected by an organ transplant, HIV/AIDS, or autoimmune diseases such as lupus.
Having a Certain Gene: If a person has a gene called, 'HLA-B 1502,' they have an increased risk of Stevens-Johnson syndrome, especially if they take certain medications for seizures or mental illness. Families of Southeast Asian, Chinese, or Indian descent are more likely to carry this particular gene.
Complications of Stevens-Johnson Syndrome
Stevens-Johnson syndrome has a number of complications associated with it. These complications may include the following:
Secondary Skin Infection or, 'Cellulitis': Cellulitis may lead to life-threatening complications, to include sepsis.
Damage to Internal Organs: It is unusual for this condition to affect a person's internal organs. Yet it might cause inflammation of the person's kidneys, liver, or heart.
Blood Infection (Sepsis): Sepsis happens when bacteria from an infection enters a person's bloodstream and spreads throughout the person's body. Sepsis is a quickly progressing, life-threatening condition that may cause shock and organ failure.
Eye Issues: The rash caused by Stevens-Johnson syndrome may lead to inflammation of a person's eyes. In mild instances, this might cause dry eyes and irritation. In severe instances, it may lead to extensive tissue damage and scarring, resulting in blindness.
Permanent Skin Damage: When a person's skin grows back after Stevens-Johnson syndrome, it might have unusual bumps and coloring; the affected person may also have scarring. Lasting skin issues may cause the person's hair to fall out and their toenails and fingernails might not grow as they should.
Testing and Diagnosis of Stevens-Johnson Syndrome
Tests and procedures used to diagnose Stevens-Johnson syndrome include a physical examination and skin test. Doctors can often identify Stevens-Johnson syndrome based upon their medical history, a physical examination, signs of the disorder and symptoms. To confirm the diagnosis, a doctor may remove a sample of the person's skin for testing in a laboratory.
Treating Stevens-Johnson Syndrome
Stevens-Johnson syndrome requires a person to be hospitalized, often times in a burn unit or in an intensive care unit. The first and most important step in treating the syndrome is to discontinue any medications that might be causing it. Due to the fact that it is difficult to determine exactly which medication might be causing the issue, a doctor may recommend that an affected person cease taking all nonessential medications. Other forms of treatment include the following:
Eye Care: People with Stevens-Johnson syndrome might see an eye specialist.
Wound Care: Wet, cool compresses help to soothe blisters while the person heals. A health care team might gently remove any dead skin and place a medicated dressing over areas that are affected.
Nutrition and Fluid Replacement: Skin loss might result in notable loss of fluid from the person's body; replacing fluids is an important part of treatment. The person may receive nutrients and fluids through a tube placed via the person's nose and into their stomach called a, 'nasogastric tube.'
Medications commonly used to treat Stevens-Johnson syndrome include pain medication, antibiotics, antihistamines and medication to reduce skin inflammation. If the underlying cause of Stevens-Johnson syndrome can be eliminated and the person's skin reaction stopped, new skin starts to grow over affected areas within a number of days. In severe instances, full recovery might take a number of months.
SJS usually begins with fever, sore throat, and fatigue, which is commonly misdiagnosed and therefore treated with antibiotics. Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips, but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. Conjunctivitis of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.
- Stevens-Johnson syndrome is more common in adults than in children.
- In the United States, about 300 new SJS diagnoses are made each year.
- Women are affected more often than men, with SJS cases occurring at a two to one ratio.
- SJS is a rare condition, with a reported incidence of around 2.6 to 6.1 cases per million people per year.
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