Steven Johnsons Syndrome
What is Stevens Johnson Syndrome (SJS)?
SJS and TEN (Toxic Epidermal Necrolysis) are potentially fatal adverse reactions to medications
such as antibiotics, anti-convulsants, anti-depressants and anti-inflammatory drugs
(prescription and non prescription such as ibuprofen). Cocaine has recently been added to the
list of causative agents and there have been reported cases related to herbal supplements containing
ginseng. In rare cases the syndrome can be caused by infection or cancer.
Effects on the Body
SJS patients have severe blistering of the skin and mucus membranes including the mouth, eyes
and genitals. When the involvement of the body is greater than 30% the classification is
changed to TEN which literally means “toxic skin death”. The blisters progress so that “sheets”
of skin are lost from large areas of the body. Huge amounts of fluids and salts seep from the raw,
exposed tissues. It’s important to emphasize that the loss of skin externally, referred to as
sloughing, is matched internally by shedding of the lining of mucosal membranes such as the mouth,
throat, lungs and gastrointestinal tract. Both stages of involvement are life threatening.
Infection at the site of exposed tissue is commonly the cause of death. Patients are extremely
susceptible to organ failure most specifically liver, lungs or heart. One example would be the
intensive mucosal shedding in the trachea and bronchial tissues leading to respiratory failure.
Treatment
The skin loss and pain levels of SJS / TEN are equivalent to chemical or thermal burns and patients
must be treated in a dedicated burn center. Discontinuation of the offending drug is vital, although
many cases are initially misdiagnosed so that the patient continues to receive the full course of the
offending medication. There is no cure for SJS and treatment is supportive only, concentrating on pain
control and prevention of infection. Recently, there has been discussion about the impact of
Intravenous Immunoglobulin (IVIG) on stopping the progression of the reaction. Trials have shown that
Amniotic Membrane will prevent serious eye damage if it can be inserted into the eyes within the first
three to five days. Unfortunately, this sight saving protocol is only being used in the most advanced
facilities, complicated by the frequent delay in diagnosis.
Surviving SJS
For those who survive, there are serious and permanent complications:
- Stricture of the Esophagus
- Asthma or C.O.P.D.
- Chronic Fatigue
- Arthritis
- Environmental and Food Allergy
- Permanent Scarring of the Skin
- Difficulty with Regulating Body Temperature as the pores are scarred shut causing overheating
- Ocular Surface Disease and Dry Eye leading to blindness
- Cardiac Diseases such as Myocarditis
Without doubt the permanent damage to the eyes and supportive ocular tissue is the most medically
challenging complication. Severe dry eye, scarring of the cornea and numerous other ocular surface
diseases are common. Frequently the eyelids become permanently grafted onto the eyeball as the "burnt"
inner eyelids heal, preventing movement of the eye or closure of the eyelids.
The formation of hard, scaly, scar tissue inside the eyelids induces intense friction on the eye,
causing blood vessels to grow into the cornea. As the cornea becomes thinner, excruciating ulcers
develop leaving permanent scar tissue. Loss of stem cells cause the cornea to become opaque,
inflammation causes cataract and diseases of the conjunctiva are progressive. Blindness can be caused
by any of these eye conditions individually, but collectively they are viewed by ophthalmologists as
THE most tortuous grouping of ocular conditions.
The severity of the dry eye has an enormous impact on the deterioration of the cornea, conjunctiva
and eyelids and is responsible for intense sensitivity to light and debilitating pain. Patients
become unable to function, spending every moment in darkness. Because of the dry eye condition,
Stevens Johnson Syndrome Survivors are not candidates for cornea transplant and face a life time
dealing with progressive and challenging inflammatory eye diseases.
For more information about dry eye click here
Before SJS
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After SJS
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SJS and Ian
Ian’s reaction was due to an antibiotic and even though he blistered progressively, he was not
diagnosed for 5 days. He suffered 5 agonizing days of feeling as though he was on fire, but was
given no pain medication. Within two hours of confirmed diagnoses he was given massive doses of
morphine and transferred to a burn unit where he was placed on life support. Loss of skin (burn)
was over 87% of his body with severe ocular involvement. Ian was fortunate to be one of the first
TEN patients to receive Intravenous Immunoglobulin
(IVIG), and as you can see from the photos, his survival can only be accredited to this intervention.
The use of amniotic membrane was not considered for his eyes. Long term sequelae for Ian involve the
lungs, eyes and skin. His immune system is severely compromised. He now has asthma and must
manage his activities and environment, in order to prevent overheating, allergies or
respiratory infection. Any future antibiotic therapy could prove fatal, and must be avoided at all costs.
Since his reaction, Ian has endured intense eye pain, light sensitivity and temporary loss of vision
in his right eye. He has had numerous surgeries to address scar tissue on the inside of his eyelids,
scarring of the conjunctiva, dense cataract and reconstruction of all four eyelids and amniotic
membrane grafting. Some procedures have had to be repeated and will be necessary again in the future.
Currently Ian wears a Boston Ocular Surface Prosthetic Lens in his right eye, and it is because of
this state of the art device, that he can function in any way.
The lens serves to keep the cornea moist while protecting it from eyelid friction (think car wiper
on a dry windshield). Even with the lens, Ian still requires eye drops every 20 minutes. It is
because of this prosthetic lens that he can attend school, and live a relatively normal life. The
inability to continue to wear this prosthetic device is unthinkable. For Ian, loss of the prosthetic
lens would mean living the rest of his life in darkness, hiding from the light and suffering
unbearable pain every minute of every day. Adult SJS survivors describe the pain as having glass
shards forced into the eye every time you blink, while at the same time, having hot needles
continually poked into the eyeball itself. Even the smallest amount of light is like having your
eyes forced open to stare into the sun on the brightest summer day. Loss of vision would be inevitable.
It is important to note that many blind people enjoy extremely happy and productive lives. However it
is the pain associated with SJS related eye trauma that renders these patients totally incapacitated,
trapped in a living hell. For this reason, it is imperative that Ian continues with his treatments
in the United States so that he can maintain his quality of life. He needs your help to do so.
Statistics and Facts
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Over 90% of SJS reactions are initially misdiagnosed as chicken pox or hand, foot and mouth disease.
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Over 5000 people are admitted to hospitals and burn units throughout USA each year with SJS.
These are new admissions only and this figure does not include patients previously admitted for
other conditions, reacting to the medications they are given as inpatients.
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Annually, there are more than 100, 000 outpatient visits for SJS in the USA, and about two million
visits for immediate hypersensitivity reactions that may be due to drugs.
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According to statistics released in 2008, There are approximately 663,000 E.R. visits in Canada
per year due to adverse reactions to medications. There are no figures available for SJS or TEN
specifically.
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In 2006, Health Canada received reports of 10,518 serious adverse skin reactions, yet estimates
that this represents less than 10% of the actual figure due to “failure to report”. If this figure
is accurate then that would convert to approximately 105,180 adverse reaction cases per year in
Canada. Again no figures available for classifications as SJS specifically.
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A 2008 study at Vancouver General Hospital has found that one in nine people in the emergency
room were there because of an adverse reaction to their medication. The study was made over a 12
week period.
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Minor instances of SJS remain largely unreported or are misclassified as Erythema Multiforme.
This puts the patient at a high risk to develop SJS or TEN, should they be exposed to the same
causative medication at any point in the future.
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SJS is one of the most debilitating adverse drug reactions recognized. It was first classified in
1922 by pediatricians A.M. Stevens and F.C. Johnson after diagnosing a child with severe ocular
and oral involvement to a drug reaction.
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Writer George Orwell was probably one of the first SJS victims. He was among the very first to be
treated with the antibiotic streptomycin. His skin fell off, his nails came off and he suffered
terribly with his eyes for the rest of his life
Conclusion
Given our own experience, and the experience of the hundreds of SJS survivors who have shared their
experiences with us, it is frustratingly obvious that education is the key. Prevention, early
diagnosis, access to state-of- the-art treatment protocols and a strict, coherent, reporting system
for adverse reactions are vital. Please join us as we work to build awareness within the medical
community and the public at large. You could save a life simply by sharing this website with your
family and friends!