Stevens Johnson syndrome (SJS) is an extremely rare disorder characterized by a severe skin reaction to medication, infection or illness.
SJS can affect people of all ages, although it is more common in children and young adults. SJS may be life-threatening and can result in a host of serious complications, such as sepsis (life-threatening blood infection) and permanent blindness. The cause of SJS is unknown, but a variety of medications and bacterial and viral infections have been associated with the disorder.
The onset of SJS is often sudden, with the appearance of multiple skin lesions. The lesions may be painful and can result in blisters and erosion of the skin and mucous membranes, especially the mouth and eyes. In some cases, areas of skin peel off with a gentle touch or pull.
SJS is usually diagnosed during a physical examination, which includes a medical history and a list of medications, particularly those taken in the last days or weeks. In some cases, a biopsy may be performed to rule out other ailments.
SJS is treated by discontinuing any medication that may be causing the reaction. Mild symptoms may be treated with moist compresses and over-the-counter medications, such as analgesics (pain relievers) and antihistamines (a medication that blocks the effects of histamine, a chemical released in body during an allergic reaction). Patients with severe skin erosions may have symptoms similar to people with burns. These patients may be hospitalized and treated in intensive care or burn units. The goal of treatment is to address symptoms and prevent infection.
Stevens Johnson syndrome has been associated with, and sometimes confused with, two other disorders with skin eruptions. Erythema multiforme is a less severe, sometimes recurrent condition. Toxic epidermal necrolysis is considered a more severe form of SJS, with many of the same symptoms.
About Stevens Johnson syndrome
Stevens Johnson syndrome (SJS) is an extremely rare skin disorder that may be caused by a severe reaction to medication, infection or illness.
Patients with SJS typically develop patchy areas of skin that may be painful and blisters in the mucous membranes, such as the mouth and eyes. In females, the vagina may be affected. Depending on the severity of the reaction, the skin may appear burned and tend to peel off.
The condition was first discovered in 1922 by pediatricians A.M. Stevens and S.C. Johnson. SJS can affect people of all ages, but is more common in children and young adults. There is usually a peak incidence of the condition in the second decade of life. In addition, the condition typically peaks in the spring and summer. SJS occurs equally among males and females.
The classification of SJS has sometimes been confused with two other conditions that also produce skin eruptions. Erythema multiforme is a less severe, sometimes recurrent condition that produces skin lesions. For years it was thought to be related to SJS, but is currently classified as a separate condition. Toxic epidermal necrolysis (TEN) produces similar symptoms to SJS, although with greater severity and skin involvement. SJS and TEN are now considered similar to each other and distinct from erythema multiforme.
Although SJS is extremely rare, it can produce serious complications, permanently affecting the skin, hair and nails. There may scarring of the skin, as well as a permanent loss of the nail beds. The lesions may eventually affect the eyes, causing permanent blindness. Inflammation from lesions may also damage the liver, lungs, heart and kidneys. Sepsis, a life-threatening blood infection, may also occur. Additional complications of SJS include photophobia (sensitivity to light), scarring of the esophagus and other mucus membranes, arthritis and chronic fatigue syndrome. Loss of fluids may also lead to shock.
Potential causes of Stevens Johnson syndrome
Stevens Johnson syndrome (SJS) is thought to be an allergic reaction. However, the exact cause of the disorder is unknown. For some cases, several medications, infections and illnesses have been associated with SJS. In other cases, no cause has been identified.
Although some medications are more often linked to SJS, the condition can result from the use of almost any drug, including over-the-counter medications. Medications that have commonly been associated with developing SJS include:
Nonsteroidal anti-inflammatory drugs (NSAIDs). Medications that do not contain steroids, and reduce inflammation and pain.
Antibiotics. Medications that kill or slow the growth of harmful microorganisms, such as bacteria.
Anticonvulsants. Medications used to treat or prevent convulsions or seizures.
Barbiturates. Medications that produce relaxation and sleep.
These medications are commonly used by millions of people worldwide. Very few people experience SJS.
Some SJS cases have been associated with various bacterial, viral and fungal infections, as well other illnesses. For instance, SJS may be related to conditions such as syphilis, acquired immune deficiency syndrome (AIDS), herpes, influenza, diphtheria, typhoid and hepatitis (inflammation of the liver). However, SJS is an extremely rare complication.
Individuals who use cocaine, and those undergoing radiation treatment for cancer, are also at increased risk.
Signs and symptoms of SJS
Stevens Johnson syndrome (SJS) may begin with a fever and other flu-like symptoms (e.g., body aches, coughing, headache) that precede the skin lesions by several days or a week. In cases associated with a medication, the symptoms typically begin within the first eight weeks of taking the drug.
The skin symptoms associated with SJS may include a red rash, skin peeling and blistering of the skin and the mucus membranes lining areas like the mouth, throat, eyes, nose, anus and genital area.
The skin lesions may be painful and have the following characteristics:
Sudden onset.
Typically develop on the face and trunk (the part of the body not including the head and limbs).
May recur or spread to other areas of the body, including the legs, arms, hands and feet.
May appear as nodules (small masses of tissue), papules (small, solid and usually inflammatory bumps on the skin) or macules (small, localized patches of discolored skin).
Appearance of a central lesion surrounded by concentric rings of paleness and redness, also called “target,” “iris” or “bull’s eye,” and which tend to be symmetrical.
Skin may exhibit Nikolsky’s sign, a condition in which the superficial layers of the skin slip free from the lower layers with a slight rubbing pressure.
Other symptoms that may be present include:
Itching (pruritus)
General sick feeling (malaise)
Joint aches
Vision abnormalities
Dry eyes
Bloodshot eyes
Eye burning, itching or discharge
Conjunctivitis
Depending on which areas of the body are affected, a number of other symptoms may occur. Blisters on the mouth may make it difficult to eat. The eyes may become painful, swollen, and in some cases, may seal shut due to pus. Scarring of the corneas (the tissue that covers the front of the eye) and photophobia (light sensitivity) may also occur.
If the urethra (the tube through which urine leaves the body) is affected, a person may experience painful urination or difficulty urinating. Blistering of the mucous membranes in the digestive tract can result in diarrhea, and difficulty breathing may occur when the respiratory tract is involved.
Individuals who suspect that they have developed SJS should contact a physician immediately. If left untreated, SJS can be fatal.
Diagnosis methods for SJS
Stevens Johnson syndrome (SJS) may be diagnosed during a physical examination that includes a medical history and list of medications, particularly those taken in the past few days or weeks.
A physician may diagnose SJS based on visual inspection of the skin lesions and other symptoms. The presence of Nikolsky’s sign, in which the top layers of the skin separate from the lower layers with a slight pressure, can indicate SJS or toxic epidermal necrolysis (TEN). For TEN, a larger area of the skin will exhibit the skin separation.
In some cases, a biopsy may be performed to differentiate SJS from other disorders. This involves removing a small sample of tissue and sending it to a laboratory where it is studied under a microscope.
Treatment options for SJS
Treatment of Stevens Johnson syndrome (SJS) focuses on addressing the underlying cause, treating symptoms and preventing infection. Medications suspected of causing SJS are discontinued.
Treatment of symptoms associated with mild cases may include:
Moist compresses applied to skin lesions.
Medications to control itching (pruritus), such as antihistamines (a medication that blocks the effects of histamine, a chemical released in body fluids during an allergic reaction).
Over-the-counter analgesics to reduce pain, inflammation, fever and discomfort.
Topical anesthetics (especially for mouth lesions) to ease discomfort that may interfere with eating or drinking.
Treatment of symptoms associated with severe cases may include:
Hospitalization in an intensive care or burn care unit. Patients with SJS “burn” from the inside out and are treated in a similar fashion to burn victims.
Replacing fluids or nutrients that may have been lost as a result of the condition.
Intravenous immunoglobulins (proteins in the blood plasma that function as antibodies) to slow or stop the allergic reaction.
Antibiotics (medications that kill or slow the growth of harmful microorganisms) and isolation from others to prevent secondary infections (e.g., sepsis).
Physicians and studies differ on the use of corticosteroids, a group of anti-inflammatory drugs that provide relief by lessening swelling, redness, itching and allergic reactions. Some studies have shown patients benefit from their use. However, corticosteroids suppress the body’s immune response, which may already be compromised by the skin damage of SJS. As a result, the use of corticosteroids may increase the risk of infection. Other studies indicated increased mortality rates among SJS patients treated with corticosteroids.
Patients with SJS may take several weeks to recover. However, this varies between patients. Individuals who received prompt treatment for the condition (within 24 to 48 hours of onset) often have a shorter duration of illness when compared to those whose treatment was delayed. Since the skin typically grows back on its own, skin grafts are not usually needed unless large areas of the body are affected.
Because the cause of SJS is unknown, there is no way to prevent the disorder. However, recognizing the symptoms and receiving immediate medical attention is the best way to lesson the severity of symptoms and minimize harm associated with the condition.
Questions for your doctor about SJS
Preparing questions in advance can help patients to have more meaningful discussions with their physicians regarding their conditions. Patients may wish to ask their doctor the following questions about Stevens-Johnson syndrome (SJS):
Do my symptoms indicate SJS?
What methods will you use to determine the cause of my symptoms?
What may have caused my case of SJS?
Does SJS pose any danger to my overall health?
What steps can I take to prevent complications from developing?
What are my treatment options? How effective are they?
Will I need to be hospitalized?
When can I expect my symptoms to subside?
What are the odds that I will develop SJS again?
Are my children at increased risk for developing SJS because I have had the condition?
