Stevens-Johnson Syndrome: Causes, Symptoms and Treatment Of The Deadly Skin Condition


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Stevens-Johnson Syndrome is a rare, albeit potentially life-threatening adverse drug reaction that often affects the skin and mucous membranes. First described in 1922 by Albert Stevens and Frank Johnson, it has been a medical emergency often requiring weeks to months of hospitalization in the intensive care or burns unit. Recently, SJS came into the spotlight in Nigeria when the Honourable Minister of Health, Professor Isaac Adewole drew public attention to two newly diagnosed cases, one of whom had already died while the other one was hospitalized at the National Hospital, Abuja. The minister consequently advised the general public to shun self-medication, a practice that is very rampant among Nigerians.

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According to a skin specialist, Dr Olanrewaju Falodun, the incidence of this deadly syndrome is high in Africa on account of extensive use of herbal preparations coupled with high HIV prevalence. It's also worthy of note that Stevens-Johnson syndrome appears to affect more women than men.

In order to stay safe from this condition, some facts you need to be familiar with are highlighted here.


1. What causes Stevens-Johnson Syndrome?

Although the exact cause of this condition cannot be identified in 25-50% of cases, self-medication and infections are the major culprits while cancers may also be responsible for few cases. Some of the drugs that have been linked with SJS include, but are not limited to common pain relievers such as paracetamol, ibuprofen and naproxen; antibiotics such as penicillin; anticonvulsants and antipsychotics as well as anti-gout medications such as allopurinol. In the same vein, infections such as Herpes simplex or zoster, HIV, hepatitis and pneumonia may cause Stevens-Johnson Syndrome.


2. Who is at risk of Stevens-Johnson Syndrome?

Any person who has had the condition in the past is at risk of a recurrence, especially if they use the same drug again. Also, if someone in your immediate family has had SJS, you may be susceptible. Similarly, persons who have viral infections such as viral pneumonia, HIV, hepatitis and herpes tend to be at a higher risk. Lastly, it has been discovered that individuals who have a gene called HLA-B 1502 may readily develop SJS if they take certain anticonvulsants or antipsychotics.


3. What are the symptoms of Stevens-Johnson Syndrome?

If you have SJS, several days before the actual rashes appear, you may develop fever, cough, runny nose, sore mouth and throat, burning sensation in the eyes as well as tiredness. The typical symptoms of SJS include red or purple itchy, rapidly spreading skin rashes that ultimately lead to blisters and ulcers on the skin and mucous membranes of the mouth, eyes, nose and genitals. The affected person may also have a swollen tongue and face as well as painful shedding of the skin.


4. How is Stevens-Johnson Syndrome diagnosed?

Stevens-Johnson Syndrome is suspected from a good history including medications used or any recent viral infections coupled with physical examination of the rashes. To confirm the diagnosis, your physician may also take a biopsy of the rash for histological analysis.


5. How is Stevens-Johnson Syndrome treated?

Immediately after hospital admission, all nonessential medications will be stopped while nutritional and fluid replacement is started to compensate for the significant fluid loss from denuded skin. Furthermore, wound care involving wet compresses to soothe healing blisters, debridement and medicated dressing is done. Other aspects of care include antitetanus prophylaxis, analgesics to relieve pain, antihistamines for itching as well as antibiotics to control infection. In the same vein, steroid creams may be applied to the skin to control inflammation.


6. What are the complications of Stevens-Johnson Syndrome?

If not promptly treated, SJS can result in secondary bacterial skin infection which may eventually spread to the bloodstream leading to sepsis, shock or organ failure. Also, the rash in SJS may lead to conjuctivitis and in severe cases, extensive tissue damage, scarring and blindness. Lastly, in rare cases, SJS has been linked to inflammation of internal organs such as the kidneys, heart, lungs and liver.

Photo credit: erythemamultiformepictures.com
 
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