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In the Know - All about… SJS (“red skin syndrome”)


In the Know

All about… SJS (“red skin syndrome”)

It almost sounds like a nonsense condition.  After all, having red skin doesn’t sound too terrible, as in: “What’s the big deal about a little sunburn?” or “So?  He has a ruddy complexion!”

But “red skin syndrome,” known mostly by its medical name of Stevens-Johnson syndrome (or SJS), is nothing to laugh at.  While very rare, it is a medical emergency that typically seems like the flu at first, but which requires hospitalization.

Stevens-Johnson syndrome is usually a severe reaction by the skin and mucous membranes to a medication or an infection.  The disease is named for Drs. Albert Mason Stevens and Frank Chambliss Johnson, U.S. pediatricians who first described the disorder in 1922.

Recovery after a bout of Stevens-Johnson can take weeks to months, depending on the severity.  And in cases caused by medication, patients will need to permanently avoid that drug and others closely related to it.

Definition and symptoms

Stevens-Johnson syndrome is defined by the skin and mucous membranes (the tiny sacs throughout that produce such body fluids as saliva and nasal mucus) undergoing a very rare and extreme allergic reaction—usually to a medication but sometimes to an infection.

In technical “medical-ese,” SJS is a mild (or milder) form of something called toxic epidermal necrolysis (TEN), or death of skin cells due to targeted poisoning.

Stevens-Johnson is estimated to affect a maximum of six out of every one million Americans a year, or 0.000018 percent of a current population of about 300 million.  The condition is more common in adults than children and in women over men.

Stevens-Johnson syndrome often begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters.  The top layer of the affected skin then dies and sheds.

Primary symptoms include:

A red or purple skin rash that spreads within hours to days

  • Facial swelling

  • Tongue swelling

  • Hives

  • Skin pain

  • Blisters on the skin and the mucous membranes of the mouth, nose, eyes and elsewhere

  • Shedding of the skin

People later diagnosed with Stevens-Johnson syndrome may experience the following symptoms several days before the primary symptom—severe skin rash—develops:

Fever

  • Sore mouth and throat

  • Fatigue

  • Cough

  • Burning eyes

Stevens-Johnson syndrome requires immediate medical attention.  Seek emergency medical care upon any of the following signs or symptoms:

  • Unexplained widespread skin pain

  • Facial swelling

  • Blisters on your skin and mucous membranes

  • Hives

  • Tongue swelling

  • A red or purplish skin rash that spreads

  • Shedding of the skin

Complications

Complications can include cellulitis or other secondary skin infection (cellulitis), which can lead to life-threatening situations like infection of the blood by bacteria (sepsis).  Other complications are inflammation of the eyes, leading to irritation and dry eyes (or worse in severe cases), or—rarely—inflammation of the lungs, heart, kidney or liver.

Causes

Stevens-Johnson syndrome is believed to be an immune system disorder, in which the immune system attacks the skin and mucous membranes.  Doctors may not be able to identify its exact cause, but it is usually triggered by a medication or an infection.

Over 100 drugs have been associated with Stevens-Johnson syndrome.  Some of the most common ones include:

  • Anti-gout medications like as allopurinol

  • Pain relievers acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve)

  • Medications to fight infection, such as penicillin

  • Medications to treat seizures or mental illness (anticonvulsants and antipsychotics)

Infections also known to have caused Stevens-Johnson in rare cases include herpes (herpes simplex or herpes zoster), pneumonia or hepatitis.

Even less commonly, radiation therapy has been known to trigger Stevens-Johnson.

Risks

Any personal or family history of Stevens-Johnson syndrome does, unfortunately, slightly increase the risk of the condition occurring (or occurring again).  In medication-related cases, taking the associated drug is highly risky in terms of recurrence.

If an immediate family member once had the condition, or a related condition called toxic epidermal necrolysis (TEN), one may be more susceptible to having the condition, too.

Also, research has found that a specific gene called HLA-B 1502 increases risk of Stevens-Johnson, especially for those taking certain drugs for seizures or mental illness.  People of Chinese, Southeast Asian or Indian descent are likelier to be carrying that gene.

Diagnosis

Tests and procedures used to diagnose Stevens-Johnson syndrome include:

Physical exam

Doctors often can diagnose Stevens-Johnson syndrome based on the patient’s medical history, a physical exam, and the disorder’s signs and symptoms.

Skin test

To confirm that diagnosis, emergency room doctors may remove a sample of skin for laboratory testing (biopsy).

Treatment

As mentioned, Stevens-Johnson is a medical emergency that must be treated immediately at a hospital—where patients will often be admitted to the intensive care unit (ICU), or a burn unit.

If seeing a doctor for a suspected case, the patient or accompanying family member should bring along all prescription and non-prescription drugs to help doctors figure out what caused the case.

Hospital treatment

The first thing doctors will do is to stop the medication or treat the infection that made the patient sick.  They’ll also try to relieve symptoms, prevent infections, and support healing.

Supportive care that the patient is likely to receive while hospitalized includes:

Fluid replacement and nutrition.  Because skin loss can result in significant loss of fluid and protein from the body, replacing those is an important part of treatment. The patient may receive fluids and nutrients through an IV and/or a tube placed through the nose and advanced into the stomach.

Wound care.  Cool, wet compresses will help soothe blisters while they heal. The hospital team may gently remove any dead skin and place a medicated dressing over the affected area(s).

Eye care.  The patient’s eyes will be kept clear, with the care team using special drops and creams as necessary to prevent them from going dry.  The patient may also need to see an eye specialist (ophthalmologist).

Medications.  Medications commonly used in the treatment of Stevens-Johnson include:

  • Pain medication to reduce discomfort

  • Medication to relieve itching (antihistamines)

  • Antibiotics to control infection, when needed

  • Medication to reduce skin inflammation (topical steroids)

 Patients will typically stay in the hospital for two to four weeks to allow for a full recovery.

Post-hospital long-term treatment

If the underlying cause of Stevens-Johnson syndrome can be eliminated and the skin reaction stopped, new skin may begin to grow over affected area(s) within several days.  In severe cases, full recovery may take several months.

The first and most important long-term step in treating Stevens-Johnson is to discontinue any medications that may be causing it.  Because it’s difficult to determine exactly which drug may be causing the problem, doctors may have patients off all nonessential medications until the specific cause is safely isolated.

Prevention

Because SJS is so very rare and because most cases are caused by medication, there’s not much one can do to prevent a first case. 

But for people at risk for carrying the one gene associated with SJS, especially those taking certain anti-seizure medications, prevention of SJS essentially consists of avoiding those medications.

Regardless of genetics, for those who’ve already triumphed over their first case, prevention essentially consists of avoiding the medication(s) that triggered their extreme allergic reaction.

It is recommended that SJS patients wear a medical alert bracelet, to notify medical personnel of their history in the rare case of another episode.

Prognosis

Any causing medication or substance (and similar items) will have to be avoided for by the patient for the rest of his or her life.  Patients may also have to contend with possible permanent bumps and discoloration of the skin; some scars, hair loss, and abnormal growth of the nails have also been known to occur.

But at the end of the day, while it can sometimes take a few weeks or even months to fully recover from a bout of SJS, virtually everyone does, and most with few if any lasting effects.