If you are living with SJS/TEN, or know someone who is, you may want to stay up-to-date on the condition as well as current treatments and news.
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Coping and Support
SJS/TEN is found to have a persisting impact on patients' lives, both physically and psychologically, including many unanswered questions and concerns. Patients are at increased risk of depression and post-traumatic stress disorder following admission to the intensive care unit. Many patients are unable to work following hospital discharge, and others may require modification to their work environment.
Patients are encouraged to speak to their healthcare provider regarding SJS/TEN should they require more education on the disease.
Diagnosis and Treatment
The diagnosis of SJS/TEN is made based upon the clinical features described above. If you or anyone you know has developed these symptoms, go to the emergency room right away or call for an ambulance.
If you are suspected of having SJS/TEN, the physician would want to know the drug that has caused the symptoms, so that it can be withdrawn as early as possible, and improve the prognosis. It’s also important that this drug is identified so that the patient is not re-exposed in the future. At their disposal, physicians will have a tool to use called the algorithm of drug causality for epidermal necrolysis (ALDEN). For patients taking multiple medications, this tool is used to determine the likelihood of which of the many drugs the patient is on is the offending one.
Physicians may also perform a routine skin biopsy so that they can examine it under a type of lighting called immunofluorescence and confirm that they have the correct diagnosis of SJS/TEN, and not a mimic of SJS/TEN. The doctor may also order other tests, such as bacterial and fungal cultures, and blood tests to determine if there are any signs of infection.
Patients with SJS/TEN are treated in the hospital, and this typically lasts 2 to 4 weeks. The management of patients requires a multidisciplinary team of healthcare providers including doctors (intensivist, specialized dermatologist, and plastic surgeon), nurses, and other professionals. The following steps will be taken by the treatment team:
- Prompt discontinuation of the offending medication that caused SJS/TEN (if it was induced by medication).
- Appropriate wound care: the skin will be kept as healthy and clean as possible by removing dead skin and applying different kinds of dressings.
- Patients will be intubated with a feeding tube to obtain proper nutrition and fluids. A great deal of fluid is lost in patients with SJS/TEN, and it is necessary to replace this fluid.
- Physicians can provide strong pain medication to help alleviate the intensity of the pain associated with this skin infection.
- Patients are at very high risk of developing an infection, and it is imperative that physicians help in reducing your risk of developing them. Sterile handling is essential in patients with SJS. Antiseptic solutions can be prepared before dressing any wounds. Antibiotics can be given to treat any infections as well.
- Medication can also be given to reduce inflammation.
The symptoms of SJS/TEN affect the skin primarily. Typically, 1-3 days before the presence of skin symptoms there are several notable signs observed, including:
- Fever (typically greater than 390C).
- Flu-like illness
- Itching or burning of the eyes
- Pain upon swallowing
- Joint pain
Symptoms that develop later on in SJS/TEN may include:
- Spots or patches of red skin that are tender to the touch. These patches can appear darker in colour in the middle, and lighter on the edges of the patch.
- Bruises on the skin can have a blister above.
- The skin of patients can blister or peel. This typically occurs on the chest, face, palms of hands, or soles of feet. These blisters can peel easily and with slight pressure of the thumb can be displaced to the side. Thus, patients must be handled with extreme care.
- On the lips, the moist inside lining of the mouth, eyes, vagina, penis, lungs and other areas, there are sores, swelling and crusting. This can cause:
- Watering of the eyes, red eye, and extreme light sensitivity.
- Different parts of the eye sticking to one another.
- Burning sensation with urinating, or difficulty urinating.
- Difficulty with breathing as lungs can build up with fluid, or lung infections can occur.
Overview: What is SJS and TEN
Overview: What is Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are two rare, but very problematic and life-threatening skin conditions. SJS and TEN cause the skin to become red and purple, cause tenderness, and cause the skin to peel away from the body. These diseases affect not only the skin on the external surface of the body, but also affect the moist lining of the mouth, nose, eyes, genitalia, and other body parts. TEN is a more severe form of SJS. The extent of skin involvement (body surface area – BSA) classifies a patient into one of three groups:
- SJS: <10% of BSA
- SJS-TEN overlap: 10-30% of BSA
- TEN: >30% of BSA
SJS and TEN have an annual incidence of 1.2–6 and 0.4–1.2 per million persons, respectively. Women are more affected than men, with a ratio of 1.5:1. The incidence of SJS/TEN will rise with age also. Patients that are more high risk are those that have HIV and patients with brain tumours that are undergoing treatment with radiotherapy and also are receiving anti-seizure medications at the same time.
SJS/TEN is mostly caused by an extremely rare side effect of medicine. Development of SJS/TEN cannot be predicted, but as mentioned previously, the likelihood rises slightly if the patient has an HIV infection. It can also occur in children with different infections.
There are over 100 drugs that have been associated with SJS/TEN. The likelihood that a patient will develop SJS/TEN from these medications is highest during the first 8 to 10 weeks of taking the medication. The following medications are most likely to cause SJS/TEN:
- Medication used to treat infections, such as:
- Nevirapine: an HIV medication, not commonly used.
- Medication that is used to treat gout:
- Medication used to prevent seizures, known as “anti-epileptic drugs”
- Medication used to treat pain, known as “non-steroidal anti-inflammatory agents”, also known as “NSAIDs”: