Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN)

What is Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)?

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, severe skin reactions that can be potentially fatal. While they are variants of the same condition, they are distinct from erythema multiforme, typically caused by infections. Key features of SJS/TEN include skin blistering, peeling, and mucous membrane involvement. Patients often experience systemic symptoms such as fever, chills, malaise, and body aches. Immediate medical attention and typically hospitalization in a burn unit are essential due to the severe nature of the condition.

What causes Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)?

SJS/TEN results from an uncommon, complex reaction to certain medications, though its exact mechanism is not fully understood. Believed to arise from an abnormal immune system response, medications or occasionally infections are the common triggers. Genetic predispositions might increase the risk of developing SJS/TEN. Notably, an HIV infection significantly elevates the risk. While it's hard to predict its occurrence, some medications have a higher associated risk, including:

• Sulfonamide, penicillin, or cephalosporin antibiotics

• Various other antibiotics

• Anti-convulsants such as lamotrigine, carbamazepine, phenytoin, and phenobarbital

• Allopurinol

• Acetaminophen

• Specific anti-viral medications, particularly nevirapine

• NSAIDs, mainly of the oxicam type

• In some rare instances, vaccinations

What are the symptoms of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)?

The onset of SJS/TEN is usually within days to a month after initiating a new medication, though anticonvulsants might take up to two months. Prodromal flu-like symptoms often precede the rash and may include fever, body aches, sore throat, red eyes, and cough. The subsequent painful red rash typically starts on the torso, spreading quickly to the face, arms, and legs. The rash can manifest as:

• Flat, diffusely red spots

• Dark purple patches

• Generalized skin redness

• Target-like lesions

• Blisters, particularly in friction areas

Mucosal areas affected can include:

• Eyes: leading to redness, pain, and light sensitivity

• Lips and mouth: presenting as red, swollen, crusted lips or painful ulcers

• Throat: potentially causing swallowing difficulties

• Genital and urinary tract: resulting in ulcers or painful urination

• Airways: leading to coughing and breathing challenges

• Bowels: potentially causing diarrhea

If you observe multiple of these symptoms post medication initiation, immediate medical consultation is imperative.

How is Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) treated?

Due to its life-threatening nature, prompt and comprehensive treatment for SJS/TEN is essential, which includes:

• Discontinuation of the suspected medications

• Hospitalization, ideally in a burn unit

• Utilization of fluid or air-cushioned beds

• Intravenous fluid replenishment

• Appropriate nutritional support

• Maintenance of optimal body temperature, often necessitating a heated room

• Pain management

• Sterile procedures

• Diligent skin care to minimize the risk of infections and further skin damage

• Addressing associated symptoms

• Considering intravenous steroids, though their use remains debated

How can Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) be prevented?

For survivors of SJS/TEN, it's crucial to abstain from the triggering medication and related drugs. If possible, they should also steer clear of:

• Anticonvulsants like carbamazepine, phenytoin, lamotrigine, and phenobarbital

• Beta-lactam antibiotics such as penicillins, cephalosporins, and carbapenems

• NSAIDs

• Allopurinol

• Sulfonamide antibiotics, including sulfamethoxazole, sulfadiazine, and sulfapyridine