Stevens Johnson Syndrome

When taking any sort of medications, there is almost always some potential for risks, side effects and complications to occur. While many of the potential side effects and complications that can arise with medications are minor or uncommon, there are others that can be very serious and have potential for mortality. Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis Syndrome (TENS), are both a form of a very serious and rare skin disorder, that could result in death, due to adverse drug reactions.

This disease affects the skin and mucous membranes, in which the layers of the skin start to peel away and separate, due to cell death. While medication reactions are the most common reason for onset, it can also occur in response to some preexisting bacterial infections. Symptoms for SJS can start off being flu-like, with a fever and a possible mild epidermal rash that can then quickly turn into severe blistering that causes the skin to be easily sloughed off.

Once SJS has affected a certain amount of skin, it may then be diagnosed as Toxic Epidermal Necrolysis Syndrome (TENS). In medical literature, SJS and TENS are the same disease, at varying levels of intensity (Foster, Foster & Foster, 2011). Many people, who have had SJS or TENS, acquired it due to an allergic reaction in response to a medication, infection or illness. However, the disease in its entirety remains to be idiopathic, and researchers have not yet found its exact cause.

Certain viruses such as influenza and hepatitis, as well as bacterial infections caused by streptococcus and mycobacteria, have all been reported to cause SJS. Penicillin’s, cephalosporin’s and sulfa drugs are all types of antibiotics that are known to be the most common onset of this disease. Other groups of medications also include analgesics, NSAIDs, antigout and psychoepileptics (Foster, Foster & Foster, 2011). Pathogenesis of SJS is not understood altogether, but according to the SJS foundation, existing evidence suggest that it is an immune-mediated disorder .

SJS can affect almost anyone. Although it is not limited to a certain geographical area, gender, or age group, the reports through the SJS foundation shows that more women and children have been diagnosed (Stevens Johnson Syndrome Foundation, 2001). People who are immunocomprimised, or are slow acetylators (person whose liver enzymes affect the metabolism of medications), unfortunately are more at risk than others (Foster, Foster & Foster, 2011). Certain leukocyte antigens that some people possess have been associated with higher risks for acquiring SJS when taking specific drugs.

This gave researchers the ability to link SJS with certain genetic factors. Symptoms for SJS may sometimes be misinterpreted as being the flu, since often times that is what the symptoms start off as mimicking. Persistent fevers, chills, sore throat and burning eyes may be the initial onset for most people with the developing disease. Since skin disorders primarily affect the outer surface layers of the body, the skin and mucous membranes will be the targeted areas for symptoms to occur as SJS continues to progress.

Blisters, rashes, hives, dermatalgia, facial edema, and dermal sloughing are the major symptoms that affect the skin in a person who is diagnosed with SJS (Mayo foundation of medical educational and research, 2011). Other common symptoms are glossitis, conjunctivitis, blepharitis, and a recent history of taking any sort of supplement, prescription or over the counter medication. These symptoms may start off seeming minute, but when the skin starts rapidly developing lesions and start to fall off, it can end up being very scary, painful and life threatening experience.

As soon as SJS arises as a possible diagnosis, it is important to immediately stop taking any of the supplements and/or medications that could have triggered it. There has yet to be a specific way to test for SJS or TENS, other than to officially diagnose it through a skin biopsy procedure. Because there aren’t any medications or specific treatments that will cure or eliminate SJS or TENS, the care is focused on solely treating the symptoms that have been brought on by them.

This will more than likely require hospitalization in a medical or pediatric ICU for patients with SJS, or a burn unit for patients with TENS, so that the proper care can be given (Mayo foundation of medical educational and research, 2011). Someone with SJS or TENS can be expected to receive IV fluids to replace the fluids and nutrition loss that occurs during the course of the disease, and wound care to prevent further complications, infections, and loss of function.

Some medications may also be given such as antihistamines to relieve itching, topical steroids to reduce skin inflammation and antibiotics to prevent sepsis or other secondary infections. Pain medications may also be administered to help alleviate some of the pain One of the most difficult things about prognosis of this SJS or TENS, is that it isn’t possible to predict during the course of the initial stage (Foster, Foster & Foster, 2011). It doesn’t become clear what the prognosis may be until the passing of time, once the disease has gotten a chance to run its course. Unfortunately, mortality rate is high, especially in patients who go left untreated and in patients who have been secondarily infected.

A way that medical practitioners determine the prognosis of mortality, is by determining the amount of Body Surface Area (BSA) of the skin has been detached. When less than 10% of the skin has been detached, the mortality rate is approximately 1-5%. This is usually the category that people with SJS fall into. TENS patients, however, have a skin detachment of more than 30%, followed by a mortality rate that is between 25% and 35% and can reach up to 50% (Foster, Foster & Foster, 2011). Many people with SJS or TENS rapidly lose large areas of skin, however in a matter of days the process can just stop.

Since new epithelial cells are being reproduced at an almost constant rate, the process of losing skin with this disease has a possibility of just stopping. At that point, it may only take approximately 3 weeks to completely regain new epithelial tissue. While SJS and TENS are very scary and painful diseases that are primarily caused by adverse drug reactions, they remain to be idiopathic. Although geneticists and researchers around the globe are conducting studies in finding out what exactly causes this disease as well as finding better and more effective treatments, they still have a long way to go before fully understanding it.

The best way to avoid getting the disease is to learn and be able to recognize the signs and symptoms, as well as taking medications cautiously, and noting the effect that each one has in the body.

Bibliography: Stevens Johnson Syndrome Foundation. (2001). Sjs fact sheet.

Retrieved fromhttp://www. sjsupport. org/fact. shtml Mayo foundation of medical educational and research. (2011, August 2). Stevens Johnsonsyndrome. Retrieved from.

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