What Is Stevens-Johnson Syndrome/TEN?
Stevens-Johnson Syndrome (SJS) is a serious skin condition in which the body's own immune system attacks the skin. As this process happens the skin begins to blister and die, after which it sloughs off. A distinguishing characteristic of Stevens-Johnson Syndrome from other skin disorders is the presence of mucosal involvement, including mouth, eyes, and other mucosal openings that blister and slough skin. SJS is characterized by 10% or less of the total body surface area being involved. In contrast, Toxic Epidermal Necrolysis (TEN) is characterized by 30% or more of the patient's body surface area being affected.
SJS/TEN is often caused by medications (most commonly medications for seizures, gout, and infection). Once the cause is identified, the medication is stopped immediately.
SJS/TEN is a medical emergency, and patients should be treated ideally, in a hospital with a burn center or where providers have expertise in dealing with this condition. Below is a hyperlink to a Journal of the American Medical Association Dermatology Patient Page reviewing what SJS/TEN is and a basic overview of diagnosis and treatment.
MOST COMMON MEDICATIONS ASSOCIATED WITH SJS/TEN
Sulfonamide antibiotics (Sulfamethoxazole/trimethoprim)
Oxicam Non-Steroidal Antiinflamatory Drugs - NSAIDs (e.g. Piroxicam)
USE OF IMMUNOMODULATORY AGENTS IN SJS/TEN
One of the puposes of Kindness for Kimberlee is to promote awareness of both the availability of and scientific evidence for the use of immunomodulatory agents in the treatment of SJS/TEN. Anecdotal information suggests that many burn units are hesitant to use immunomodulatory agents in treating SJS/TEN.
It should be noted that much of this data is observational and limited to case reports or case series. However due to the rarity of SJS/TEN, it is unlikely that there will be high quality evidence available in the near future.
Most commonly utilized immunomodulatory agents for SJS/TEN are corticosteroids, intravenous immune globulin (IVIG), cyclosporine, infliximab, and etanercept, among others. Use of immunomodulatory agents has the potential to revolutionize the treatment of SJS/TEN. Below is a link to articles or PubMed references about use of immunomodulatory agents in SJS/TEN.
Zimmermann S, Sekula P, Venhoffetal M. Systemic immunomodulating therapies for Stevens-Johnson syndrome and toxic epidermal necrolysis: a systematic review and meta-analysis. JAMA Dermatology. 2017;153:514–522.
González-Herrada C, Rodríguez-Martín S, Cachafeiro L, et al. Cyclosporine use in epidermal necrolysis is associated with an important mortality reduction: evidence from three different approaches. J Invest Dermatol. 2017;137:2092–2100.