What are some particular medications that trigger this severe skin condition? What are the clinical features of SJS/TEN? What kind of treatment are available?
top of page
Per vedere come funziona, vai al tuo sito pubblicato.
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)
24 commenti
Mi piace
24 commenti
bottom of page
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe skin hypersensitivity reactions most often triggered by medications. Stevens-Johnson syndrome is a less severe version of toxic epidermal necrolysis. Although clinically similar, the distribution and extent of hypersensitivity allow us to differentiate between them. SJS affects less than 10% of the body surface area, whereas TEN affects more than 30% of the body surface area. Both conditions are rare but life-threatening, affecting roughly 2 people per million people each year. There are varying forms in which SJS/TEN can present itself, however, early detection is ideal upon noticing tiny maculopapular and vesicular lesions to the extent of bullous lesions.
Several drugs can trigger such a reaction. In fact, “drugs precipitate over 50% of SJS cases and up to 95% of TEN cases”. These drugs include sulfa drugs, penicillins, fluoroquinolones, cephalosporins, anticonvulsants, NSAIDs, antiretrovirals, and others. In non-drug-induced cases, severe infections, vaccines, and graft-versus-host disease can induce a reaction. Studies have been conducted to determine typical mortality rates. In 2023, it was reported that SJS mortality rates ranged from 19.4% to 29%, and TEN mortality rates ranged from 14.8% to 48%. Once SJS progresses to TEN, massive bullae formation occurs, resulting in ruptures and peeling of the skin. An estimated 70% of individuals who heal are left with scars.
Although the exact mechanism is unknown, it is believed that drug-induced SJS/TEN is Type IV cell mediated. The body’s T cells initiate an inflammatory response to the foreign antigen. CD8+ T cells have been identified as mediators of blister formation in TEN. It is suggested that granulysin is released from cytotoxic T cells and natural killer cells, resulting in keratinocyte death, creating blister fluid. Due to the severity of the hypersensitive reaction, it is essential to discontinue any causative agents/drugs and take the patient to a hospital. Before starting any offending agents such as carbamazepine or allopurinol, HLA genetic testing can be conducted to prevent SJS/TEN whenever possible.
Within 3 weeks of starting a causative drug, patients develop symptoms such as fever, headache, cough, and keratoconjunctivitis. This is followed by macules appearing on the face, neck, and trunk of the body. As the disorder progresses, large bullae and sloughing can be seen. The skin and mucosal membrane get damaged, affecting the body’s thermoregulatory ability. Fluid replacement is one of the key factors in treatment to regulate and maintain the body’s temperature due to its impaired ability. As several components of the body have now been damaged, each must be carefully attended to.
Non-NSAID painkillers and oral and topical anesthetics are essential in pain management, morphine being a commonly administered, controversial drug. Mouthwashes such as chlorhexidine and topicals such as silver nitrate aid in repairing the damaged membrane and skin barrier, followed by dressing the wounds, reducing the introduction of infections. SJS/TEN treatment goes as far as providing lung care with aerosols and physiotherapy. Many patients are given psychiatric support due to the long-lasting effects of the individual's quality of life. Alongside these obvious treatments, there have been reports of patients benefiting from adjunctive treatment with cyclosporine, intravenous immunoglobulin, plasmapheresis, and systemic corticosteroids, although their roles remain controversial. An aggressive skin disorder, SJS/TEN requires patients to undergo specialized care and routine reassessments to best treat their conditions.
References
Benedetti, J. (2024, February 21). Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) - dermatologic disorders. Merck Manuals Professional Edition. https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten
Mayo Foundation for Medical Education and Research. (n.d.). Stevens-Johnson syndrome. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/stevens-johnson-syndrome/symptoms-causes/syc-20355936
U.S. National Library of Medicine. (n.d.). Stevens-Johnson Syndrome/toxic epidermal necrolysis: Medlineplus Genetics. MedlinePlus. https://medlineplus.gov/genetics/condition/stevens-johnson-syndrome-toxic-epidermal-necrolysis/
Labib A, Milroy C. Toxic Epidermal Necrolysis. [Updated 2023 May 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK574530/
Hasegawa A, Abe R. Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis. F1000Res. 2020 Jun 16;9:F1000 Faculty Rev-612. doi: 10.12688/f1000research.24748.1. PMID: 32595945; PMCID: PMC7308994.
Harr T, French LE. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet J Rare Dis. 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721; PMCID: PMC3018455.
Lee HY, Walsh SA, Creamer D. Long-term complications of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN): the spectrum of chronic problems in patients who survive an episode of SJS/TEN necessitates multidisciplinary follow-up. Br J Dermatol. 2017 Oct;177(4):924-935. doi: 10.1111/bjd.15360. Epub 2017 Sep 22. PMID: 28144971.