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What is Stevens-Johnson Syndrome?

January 4, 2021

Stevens-Johnson syndrome is a life-threatening condition characterized by severe skin rash, blisters in the mucous membranes and shedding of the skin. The disease is very serious and can result in lasting complications and death. A more severe form of the same condition is toxic epidermal necrolysis.

Symptoms of Stevens-Johnson Syndrome

Stevens-Johnson syndrome and toxic epidermal necrolysis are associated with painful SJS symptoms that affect the skin and mucous membranes. Symptoms include:

  • Reddish-purple rash
  • Sore throat
  • Sores in the mucous membranes, typically in the mouth, eyes, nose and genitals
  • Shedding of the skin (referred to as sloughing)

Patients will also experience fever, body aches, chills and fatigue. When the rash affects 10 percent or less of the body surface, it is Stevens-Johnson syndrome. It becomes classified as toxic epidermal necrolysis when it spreads to more than 30 percent.

Complications of Stevens-Johnson Syndrome

Both forms of the disease are life threatening and must be treated immediately. The active stage of the blistering and shedding of the skin may last from one to 14 days. During this time, the skin will die and peel off. A person affected by this condition will lose large amounts of water and salt through the loss of skin and can suffer dehydration. He or she will likely be treated at a burn unit, and will be given fluids intravenously.

The wounds are painful and are susceptible to infection. Cool, wet compresses will be applied to soothe the area and antibiotics will likely be administered to fight off infection. Severe infection can lead to death.

Patients are at risk of suffering long-term side effects from both Stevens-Johnson syndrome and toxic epidermal necrolysis. Some potential complications include severe scarring, permanent blindness and organ failure.

Causes of Stevens-Johnson Syndrome

The leading cause of Stevens-Johnson syndrome is a severe reaction to a medication. Many cases have been linked to the following types of drugs:

  • Sulfa antibiotics
  • Barbiturates
  • Anticonvulsants
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)

Stevens-Johnson Syndrome Symptoms

Stevens-Johnson Syndrome (SJS) is a rare hypersensitive skin reaction, usually to certain drugs. When someone first has the condition, they may feel like they're coming down with the flu. Flu-like symptoms will soon be followed by a severe skin rash and blistering of the mucous membranes.

Stevens-Johnson Syndrome Early Symptoms

The first symptoms of Stevens-Johnson syndrome create no reason for alarm. They usually appear between one and three weeks after taking the medication causing the condition. Flu-like symptoms might include:

  • Body aches
  • Fever
  • Sore throat
  • Headache
  • Cough
  • Conjunctivitis

Specific Symptoms of Stevens-Johnson Syndrome

These early symptoms are followed by a fast-spreading red and purple rash, similar in appearance to a bull's-eye. The rash will typically cover the upper body, face and neck and may appear other places on the patient's body. These bull's-eye-like marks on the skin

  • Spread
  • Merge
  • Grow large
  • Blister
  • Fall off

This skin is painful and peels off within one to three days.

Once these symptoms appear, their configurations, the rapidity and course of their spread and the blistering and sloughing of the skin tip the doctor off to the diagnosis. A cellular examination of the skin reveals that it is dying and this can confirm the diagnosis.

Stevens-Johnson syndrome is a at one end of a spectrum of hypersensitive skin reactions. The same disorder, when it becomes much more serious and lethal, is called toxic epidermal necrolysis. Together these conditions affect from one to five persons per million.

Treating Stevens-Johnson Syndrome Patients

Patients will usually require the type of care similar to the care needed by burn patients. They may be hospitalized in a burn unit. Great attention must be given to avoid infection, because the sloughing of the skin opens the body up to this risk. Fluids and salts are given intravenously.

Stevens-Johnson syndrome may occur more frequently in patients receiving bone-marrow transplantsHIV patients with certain infectionssystemic lupus erythematosus sufferers, and those with other chronic rheumatologic illnesses.

Drug Reactions Cause More Than 50 Percent of Cases

More than half of Stevens-Johnson syndrome patients get the disorder as a result of a drug reaction. Drugs reported to be among the more common sources of the disease are:

  • Sulfa drugs
  • Other antibiotic medications
  • Drugs to treat epilepsy
  • Miscellaneous other drugs

Over-the counter drugs such as Advil® and Motrin® also have been reportedly linked to Stevens-Johnson syndrome.

Other causes of Stevens-Johnson syndrome are believed to be:

  • Graft-vs.-host disease
  • Infection (a particular type of lung infection or a certain kind of pneumonia)
  • Vaccination

In rare instances, no cause can be found.

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are two versions of the same disease, distinguished from each other by severity. Both are hypersensitive life-threatening skin reactions, usually to a drug, that causes the skin to:

  • Form a severe rash
  • Blister
  • Peel
  • Shed

People who experience Stevens-Johnson syndrome or toxic epidermal necrolysis will also develop painful sores in the mucous membranes of the:

  • Mouth
  • Ears
  • Eyes
  • Nose
  • Genital area

During the beginning stages, a patient will usually feel very sick and the affected areas of the skin are painful. Before the outbreak on the skin, a patient may experience fever, cough, sore throat body aches, chills and other flu-like symptoms.

When Stevens-Johnson Syndrome Becomes Toxic Epidermal Necrolysis

The two disorders are different versions of the same disease at opposite ends of a spectrum of severity. When 10 percent or less of the body area is affected, the disorder is called Stevens-Johnson syndrome. When 30 percent or more of the skin is affected, it is considered toxic epidermal necrolysis (TEN). If between 10 and 30 percent of the skin is involved, it is considered to be an overlapping SJN-TEN syndrome.

The skin in both levels of the disease will blister, peel and fall off. People with toxic epidermal necrolysis will experience the symptoms over larger areas of the body, and the skin may begin to peel off in sheets (otherwise known as sloughing). The death rate is about 7.5 percent in children and 20 to 25 percent in adults. The prognosis depends upon how early the Toxic Epidermal Necrolysis diagnosis is made and treatment begins.

Because a patient's body reacts similarly to the body of a burn victim, treatments will likely be done in a hospital burn unit, where the patient receives fluids and salts intravenously and the skin is scrupulously cared for to prevent infection.

Causes of Toxic Epidermal Necrolysis

According to reports, Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are almost always caused by a severe allergic reaction to a drug, usually a sulfa antibiotic. Drugs cause more than 50 percent of the Stevens-Johnson syndrome cases, and as much as 95 percent of toxic epidermal necrolysis cases. Aside from sulfa drugs, other drugs known to cause Stevens-Johnson Syndrome are:

  • Other antibiotics
  • Some over-the-counter nonsteroidal anti-inflammatory drugs
  • Anticonvulsant medications
  • Barbiturates
  • A drug to treat gout

AIDS patients are said to be at a higher risk of developing the skin disease as well as others with compromised immune systems. In addition to drugs, infections can lead to the disorders, including:

  • Herpes (herpes simplex or herpes zoster)
  • Influenza
  • HIV
  • Diphtheria
  • Typhoid
  • Hepatitis

Some vaccinations, graft-vs.-host diseases, and radiation therapy or ultraviolet light have also been reported to cause the disorders.

Stevens-Johnson Syndrome and the Skin

Stevens-Johnson syndrome is the term used to describe a rare, potentially fatal disorder that involves the skin and mucous membranes. Patients who suffer from the condition will likely suffer from flu-like symptoms before it shows itself as a rash on the skin. Once the rash appears, it is important to seek medical treatment immediately, as the condition spreads rapidly and may have lasting effects.

How Stevens-Johnson Syndrome Affects the Skin

People who have Stevens-Johnson syndrome will develop a painful rash with a purplish color. The rash may start out on the face and trunk, but will spread rapidly if not treated properly and will begin to form blisters. Once the skin blisters, it will die and eventually begin to shed off. Another common symptom of the syndrome is the formation of blisters in the mucous membranes—usually the mouth, nose and eyes.

Toxic Epidermal Necrolysis and the Skin

A much more severe and deadly version of Stevens-Johnson syndrome is Toxic Epidermal Necrolysis. The condition is called Toxic Epidermal Necrolysis once it affects more then 30 percent of the skin on the body. Patients with this more severe form of the condition will usually experience skin shedding over large portions of their bodies. The skin may come off in large sheets.

Treating the Skin

Patients are typically treated in a burn unit, because the damage to their skin is similar and there they will receive the best care.

More Information on Stevens-Johnson Syndrome and the Skin

For more information about how Stevens-Johnson syndrome affects specific areas of the body, please select from the following:

  • Stevens-Johnson Syndrome and Rash
  • Stevens-Johnson Syndrome and Blisters

Specific Areas on the Skin & Mucous Membrane:

  • Arms
  • Back
  • Legs
  • Mouth
  • Eyes

Stevens-Johnson Syndrome and the Arms

Stevens-Johnson Syndrome is a potentially life-threatening allergic reaction of the skin and mucous membranes, most often to a drug. It is one of the most debilitating adverse drug reactions known. Often, the rash begins on the feet and hands, and then spreads to the arms, legs and face.

The syndrome is named for the two pediatricians who in 1922 discovered it — A.M. Stevens and S.C. Johnson.

The reaction first appears as a flat, splotchy red and purple rash that:

  • May resemble a target
  • Blisters in the center
  • Becomes raised
  • Sloughs off

In addition to the arms, hands, feet, legs and face, the illness also involves the mucous membranes, including the eyes, ears, mouth, nose, genital areas, anus, and urethra.

Toxic Epidermal Necrolysis (TEN)

People often get the disorder confused with toxic epidermal necrolysis (TEN). Toxic epidermal necrolysis is a term used to describe the same condition, only at a much more dangerous and severe state. The illness is considered to be SJS when it covers about 10 percent of the body or less. When it spreads to 30 percent or more of the skin surface, it is then labeled TEN. If between 10 and 30 percent of the body is affected, it is considered to be an overlapping syndrome.

The skin comes off of the two types of patients in different degrees.

  • SJS patients lose their skin by small areas of peeling and flaking
  • TEN patients lose their skin in sheets covering larger areas of the body

Before the rash and lesions develop, patients will probably feel ill with flu-like symptoms such as fever and body aches. They continue to feel very sick and in pain as the symptoms progress.

Causes of the Skin Disorder

Stevens-Johnson Syndrome is most often a result of a severe allergic reaction to a medication by drugs, usually a sulfa antibiotic. Other antibiotics and kinds of drugs reported to have caused this type of reaction include:

  • Nonsteroidal anti-inflammatories (Children’s Advil and Motrin in particular)
  • Anti-seizure medication (especially Dilantin to treat epilepsy)
  • Drugs to treat gout
  • Penicillin and other antibiotics
  • Some vaccinations
  • Barbiturates
  • Recent cases of SJS have been attributed to cocaine, mirtazapine (an antidepressant), and the anti-tumor drugs adalimumab, infliximab, and etanercept

Some brand names you may recognize include:

  • Children's Motrin®
  • Children's Advil®
  • Children's Tylenol®
  • Dilantin®
  • Bextra®
  • Daypro®
  • Diclofenac®
  • Dolobid®
  • Flagyl®
  • Lamictal®
  • Naprosyn®
  • Neurontin®
  • Remicade®
  • Topamax®

Infections, such as herpes simplex and herpes zoster, may also cause SJS. So can influenza, hepatitis, HIV, typhoid, and diphtheria. Because the illness affects the body in much the same way as burns do, patients who experience the rash on their arms, or in other parts of the body will likely be treated in a burn unit. Patients are at a very high risk of infection when the would are open, and are said to be under the best care in a burn unit.

Stevens-Johnson Syndrome and the Back

Stevens- Johnson Syndrome (SJS) is a rare, but severe life-threatening allergic skin reaction to some substance, most often a medication. Painful lesions of Stevens-Johnson syndrome (SJS) usually begin on the palms, soles of the feet, the face and the trunk. Then they spread to the back, the abdomen and the extremities.

When the lesions occur on the back, and in particular if they spread to cover most of the surface area of the back, the illness is more likely to be a severe form of the disorder because of the large area of the body covered. If the skin on the back peels off in sheets rather than flaking off in smaller patches, the syndrome may be toxic epidermal necrolysis (TEN), a more severe form of Stevens-Johnson Syndrome.

SJS and TEN are on different ends of the spectrum of the same disorder. SJS is defined as covering less than 10 percent of the body. But, when 30 percent or more of the body is involved, the disorder is considered to be toxic epidermal necrolysis. Both forms of the illness are life-threatening and survival is best when the Stevens-Johnson Syndrome diagnosis is made early so treatment can begin.

Stages of Progression

When the syndrome appears on the back, the following stages will most likely occur:

  • A flat red and purple rash appears, often in the shape of a target
  • The rash becomes raised and blisters
  • The blisters go from being solid fluid filled masses to softer lesions that eventually peel off

At this time, blisters may also form on mucous membranes of the mouth, throat, eyes, ears, nose, genital area, and the anus. The face and tongue will probably be swollen.

When the back is affected, patients will most likely need to be treated similar to those who have been burned. Cool compresses may be laid over the affected area on the back to provide relief. It is very important for caregivers to keep the wounds clean to prevent any type of infection from occurring. Antibiotics will likely be prescribed to prevent infection.

Stevens-Johnson Syndrome and the Eyes

The eyes of patients with Stevens-Johnson Syndrome (SJS) can be severely affected. The most serious consequence for the eyes is blindness. SJS can produce a number of other Stevens-Johnson Syndrome symptoms in the eyes. These include:

  • Tearing
  • Red eyes
  • Dry eyes
  • Painful eyes
  • Feelings of a foreign object in the eye
  • Burning
  • Extreme sensitivity of the eyes to light
  • Decreased vision
  • Itching and scratchiness
  • Twitching eyelids
  • Double vision
  • Puffiness of eyelids

SJS affects the skin and mucosal tissues. Of the mucosa, the most commonly affected are the mucous membranes of the mouth. Next, occurring in half of all Stevens-Johnson Syndrome patients, is the swelling and inflammation of the conjunctiva (the clear mucous membrane covering the eyeball and the inner part of the eyelid.)

Patients who have severe scarring of the cornea from Stevens-Johnson Syndrome can suffer permanent blindness after the illness has passed. If you or a loved one is blind, or has permanent vision damage after a case of Stevens-Johnson Syndrome, you may qualify for a financial recovery that would help you deal with the damages you’ve suffered and the challenges you now face. Financial compensation may help with special medical equipment to help with the condition, including:

  • Reading software for the blind
  • Portable scanning and reading software
  • Scan-talking devices
  • Technology, such as talking clocks
  • Canes, wheelchairs and more

There are many advancements for people who are blind. Financial compensation will ensure our clients are able to obtain the latest products to ensure the highest quality of life possible. Money will also help with other financial losses such as lost wages and a decreased earning capacity if damage to the eyes prevents a patient from pursuing a normal course of employment.

Stevens-Johnson Syndrome and the Mouth

Stevens-Johnson Syndrome, a rare and life-threatening allergic reaction of the skin, can begin with visible lesions only around the mouth. The condition affects the body’s skin and mucous membranes, and could damage the organs and lead to death. When the syndrome first targets the mouth there can be lesions on the lips as well as blisters inside the mouth, the cheeks and the throat. The face and the tongue swell.

The disorder is extremely painful. When the skin on the body becomes involved, it begins with a red and purple flat rash, sometimes in the shape of a target. The rash becomes raised and blisters, causing the skin to slough off. At this point, the body is literally open to severe infection.

Blisters may occur on:

  • The mouth and throat
  • Ears
  • Nose
  • Cornea and tissue around the eyes
  • Rectum
  • Genitalia
  • Urethra

A person may have a fever, sore throat, headache and other flu-like symptoms before the blisters and lesions appear on the body.

Reaction to Drugs

Drugs — usually sulfa antibiotics — have been reported as the number one cause of Stevens-Johnson Syndrome. But other antibiotics; nonsteroidal anti-inflammatories, such as Children’s Advil® and Motrin®; medication to treat epilepsy, especially Dilantin®; barbiturates; and drugs to treat gout have also been named in SJS cases.

Additional causes include some vaccinations, some infections and exposure to radiation and ultraviolet light. Persons with compromised immune systems, such as patients with AIDS or systemic lupus erythematosus are at increased risk of getting SJS.

People with SJS have a life-threatening illness and should be treated in an intensive care burn unit in a hospital. In addition to caring for the wounds, the most important aspect of the hospital care is to prevent infection that could enter the patient’s system through the open wounds that are left when the skin falls off. The affected areas of the skin must be kept scrupulously clean.

Care givers in the burn unit gently remove flaking skin. They cover the lesions and open areas of skin with soothing compresses to promote healing and alleviate pain. Fluids and nutrients are given administered in the vein. Patients may heal completely, but also may be left with permanent damage to their bodies.

Severe complications can include:

  • Eye problems, including blindness, caused by scarring of eye tissue
  • Scars, discoloration and bumps on the skin
  • Sepsis, a massive infection that progress quickly through the bloodstream and can lead to shock and organ failure
  • Cellulitis in addition to SJS. The acute skin infection can cause meningitis
  • Serious damage to the lungs, heart, kidneys and liver

Stevens-Johnson Syndrome Complications

Early diagnosis and treatment are especially important to patients with Stevens-Johnson Syndrome (SJS). Just as in patients with severe burns, fatal complications can result from the disorder if treatment is delayed for too long.

Stevens-Johnson Syndrome is usually caused by a sulfa drug, but can also be caused by other antibiotics and medications. In rarer instances, infection, a weakened immune system, or exposure to an external physical event such as radiation or ultraviolet light may lead to Stevens-Johnson Syndrome.

Complications of Stevens-Johnson Syndrome

Even with survival, the patient may take weeks and even months to recover. Aside from death, other complications of the rare but serious disorder include:

  • Cellulitis (a secondary skin infection) that can produce life-threatening complications; One of these is meningitis, an infection of the membrane and fluid around the spinal cord and brain
  • Sepsis, which is a body-wide infection of the bloodstream that can quickly lead to shock and organ failure
  • Dry eyes or in more severe situations severe damage of the eye tissue, scarring, and permanent blindness
  • Hypersensitivity of the eyes to light
  • Lung damage
  • Chronic obstructive pulmonary disease
  • Internal organ damage to the heart, kidneys, and liver
  • Asthma
  • Permanent damage to the skin resulting in bumps, scars, and abnormal pigmentation
  • Permanent loss or disfigurement of the finger and toe nails
  • Hair loss
  • Chronic fatigue syndrome
  • Arthritis
  • Scarring and damage to the esophagus which causes problems with eating and drinking

The symptoms of Stevens-Johnson Syndrome are similar to those of the flu in the first stage of the illness. They usually occur within three weeks of taking an offending drug. These include fever, chills, body aches, sore throat, and cough. The Stevens-Johnson Syndrome patient feels terrible.

Skin Complications

A red to purple rash, sometimes in the shape of a target, develops on the skin and the mucous membranes (mouth, ears, eyes, genital area and other mucous membranes internally). The blotches of the rash merge and blister, and the skin sloughs off. In a more serious version of the disorder, called toxic epidermal necrolysis, the skin sheds or can be pulled off in sheets.

Patients with Stevens-Johnson Syndrome need to see a doctor and be diagnosed as soon as possible, so they can begin treatment. Just like patients who are burned, Stevens-Johnson Syndrome patients will be treated with intravenous fluids, sometimes certain medications, and scrupulous treatment of the affected areas of the skin to prevent infection.

If a drug reaction is the cause, the patient must stop taking the medication at once.

Stevens-Johnson Syndrome Treatment

People who are diagnosed with Stevens-Johnson syndrome will most likely need to be admitted to the hospital. And, in many cases, they will be admitted to the burn unit where they can receive the proper care. Especially in severe cases of Stevens-Johnson syndrome, and in cases that turn into Toxic Epidermal Necrolysis, patients will lose high volumes of fluids and salt through all the lost skin. The damage puts patients at a high risk of organ failure and severe infection. The damage may be similar to that of a burn patient.

Drugs and medications that are suspected of causing a severe allergic reaction will be  discontinued. Stevens-Johnson Syndrome patients will receive fluids and salts through the veins and will be monitored closely.

Treating the Blisters

The painful blisters and damaged skin may be treated with one or more of the following:

  • Continual application of ointments
  • Antibiotic and steroid drops
  • Cool compresses applied for soothing the skin

Medications to Treat Stevens-Johnson Syndrome

Some medications may be used in the treatment of Stevens-Johnson syndrome, such as:

  • Pain medications to help ease the pain
  • Antihistamines to help with the itching
  • Antibiotics to help prevent infection
  • Topical steroids to help with the inflammation of the skin

In the most severe cases, skin grafting may be used to treat large portions of the skin that has been damaged. Skin grafting is the term used to describe a procedure in which healthy skin from one part of the body is removed and attached to the damaged area. This process is rarely used, but can help with the healing process.

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