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 I
will never forget the first patient of mine that developed Stevens-Johnson
syndrome - we'll call her Mrs. M. She came into the clinic with
a urine infection that required antibiotics. I wrote her a prescription
and advised her to take the full five-day course. She agreed to
do so and thanked me with a smile.
Three
days later, Mrs. M showed up in the emergency room on a night I
was on call. She had developed large blisters over most of her body
and was very ill. Though I had never had a patient with the condition
before, I recognized the development of Stevens-Johnson syndrome
right away from the textbooks I had studied. I also knew it was
a result of the antibiotic I had prescribed her. Before I could
tell Mrs. M what had happened to her, she looked up at me, winked
and said, "I took the medicine just like you asked, don't worry!"
Though
Mrs. M nearly broke my heart with her comment, side effects are
an unfortunate problem with every prescription medication. Stevens-Johnson
syndrome is a fairly uncommon disorder than can result from medications,
infections, and even vaccines. Often, the syndrome occurs for unknown
reasons.
What
is Stevens-Johnson syndrome?
Stevens-Johnson
is usually classified as a severe form of a skin rash known as erythema
multiforme, although it is unknown if the two are truly related.
The hallmark of Stevens-Johnson syndrome is the development of large
blisters in the mouth, in the throat, on the skin, around the anus
or genitals, or even on the eyes. These blisters may rupture, leaving
the immature layers underneath exposed, which may cause severe pain
or discomfort. Eye inflammation can be severe and may result in
scarring and even blindness.
In
addition, affected persons may develop reddish skin rashes in various
shapes, sizes, and locations in addition to joint pains, fever,
and itching. A wide range of distribution and severity of these
symptoms may occur, sometimes making the diagnosis of Stevens-Johnson
syndrome difficult.
What
causes it?
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The
most recognizable cause of Stevens-Johnson syndrome is medications,
as the time course so often
is suggestive in this setting. If a person is prescribed a drug
and develops a skin rash within two weeks of when they start taking
the drug, the cause is presumed to be the medication. If you develop
a rash after starting a new medication, stop taking the drug,and
call your doctor immediately.
The
most common drugs that cause Stevens-Johnson syndrome are the penicillins
(e.g., amoxicillin, ampicillin, penicillin, Augmentin), sulfa drugs
(e.g., Bactrim - the drug I prescribed for Mrs. M) and barbiturates.
This
does not mean that you should refuse to take these medications.
Stevens-Johnson syndrome occurs in less than 1 out of every 2000
people who take a
form
of penicillin, the most likely drug to cause this reaction. However,
mild rashes develop in roughly one percent to two percent of people
who take penicillin.
Once
a person develops a reaction to a medication, they should not take
that medication or similar medications again unless absolutely needed
for a life-threatening condition.
Another
cause of Stevens-Johnson syndrome is infections. The most common
culprit is the Herpes simplex virus, although it is also associated
with some of the bacteria that cause pneumonia, as well as other
viruses. Vaccines are a very rare cause of the syndrome.
How
is it treated?
Treatment
of Stevens-Johnson involves stopping any recently started drugs,
treating any underlying infections, and tending to the affected
areas of the body.
Areas
of skin where blisters have ruptured leave open sores on the skin,
which can become infected. Persons with large areas of skin involvement
are usually admitted to the hospital for treatment of the skin with
cleaning, topical antibiotics, and dressings.
Observation
for worsening symptoms, intravenous fluids and systemic antibiotics
or anti-inflammatory medications may also be required. Itching can
also be treated with medications to relieve this annoying symptom.
Most
cases resolve on their own whether or not a cause is found. Skin
biopsies may be required to make a diagnosis in difficult or atypical
cases. In rare instances, death can occur from a severe case due
to widespread skin involvement and infection.
Though
I have seen more cases since that time, Stevens-Johnson syndrome
will always make me think of Mrs. M. Thankfully, she recovered without
consequences and forgave me, and the medication I prescribed. I
make her tell me the name of the medication she is not allowed to
take whenever I see her - just in case
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