SJS/TEN steven johnson syndrome skin allergic reaction to medication medicine RX prescription drug
Palmieri TL, Greenhalgh DG, Saffle JR, et al. A multicenter review of toxic epidermal necrolysis treated in U.S. burn centers at the end of the twentieth century. J Burn Care Rehabil. 2002;23(2):87-96. doi:10.1097/00004630-200203000-00004
Introduction: The diffuse epidermal exfoliation seen in Steven Johnson Syndrome (SJS) and To
Palmieri TL, Greenhalgh DG, Saffle JR, et al. A multicenter review of toxic epidermal necrolysis treated in U.S. burn centers at the end of the twentieth century. J Burn Care Rehabil. 2002;23(2):87-96. doi:10.1097/00004630-200203000-00004
Introduction: The diffuse epidermal exfoliation seen in Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) is similar to skin loss in second degree burns, and many of these patients are referred for treatment at burn centers. Treatment can differ markedly from center to center, and mortality can range from 25% to 70%, including a considerable morbidity. However, our experience over a 15-year period from 2000 to 2015 with 40 patients found a mortality rate of only 10% (4/40). The purpose of this paper is to discuss our treatment algorithm as a model for other centers treating SJS/TENs patients.
Methods: Records were reviewed for all patients admitted to the LAC+USC burn unit between 2000 and 2015 and 40 patients were identified with biopsy-proven SJS or TENS.
These cases were reviewed for age, gender, initial and greatest TBSA, causative drug, pre-existing medical conditions, and morbidity and mortality. All data were entered into the SPSS statistical software package and all statistical analyses were performed using this program.
Results: Our treatment algorithm focused on early referral to a specialty burn unit, immediate discontinuation of the offending drug, fluid resuscitation, nutritional supplementation, and meticulous wound care.
Average time to transfer to a burn unit was 3.36 days. Silver-releasing antimicrobial dressings were applied to the affected skin surface and changed every 3 days. Mupirocin coated petroleum gauze was used for facial involvement. Steroids were tapered and discontinued if initiated at an outside facility (58% of patients), and starting after 2001, all patients received a course of IVIG.
All patients received fluid resuscitation, and the majority received supplemental tube feedings (69%). Average length of total stay was 17.1 days and length of ICU stay 15.9 days. While 44% were transferred to another facility for further rehabilitative care, 37% of patients discharge to home. In patients discharged home with complete resolution of skin lesions, time to healing was an average of 14 days.
Discussion: With our 10% mortality rate in 40 patients, our study represents a relatively large study population while maintaining a relatively low mortality rate. The demographic data from our study largely aligns with the existing literature, and we therefore feel that our low mortality rate is due to our treatment algorithm, rather than to a less severe pathology in our patient population.
This claim is supported by a standard mortality ratio of 1.68. This ratio proves a significantly improved mortality than would be expected based on disease severity on admission.
Keywords: Steven Johnson Syndrome; Toxic Epidermal Necrolysis.
The study by Clark et al. (2021) in JAAD International investigates the impact of delayed admission to a specialist referral center on the mortality rates of patients with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The research found that delayed admission (defined as 5 days or more after the onset of skin blist
The study by Clark et al. (2021) in JAAD International investigates the impact of delayed admission to a specialist referral center on the mortality rates of patients with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The research found that delayed admission (defined as 5 days or more after the onset of skin blistering) significantly increases mortality rates compared to early admission (within 4 days).
Specifically, the mortality rate for delayed admissions was 37%, compared to 14% for early admissions1.
To the Editor: Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare, severe cutaneous adverse drug reactions. The average mortality across the spectrum is 25%.
1. Guidelines recommend that such cases be managed in reference centers.
2. Our study aims to evaluate the impact of
delayed admission to an SJS/TEN referral center on clinical outcomes.
A retrospective cohort study was conducted from 2005 to 2018 at the Singapore General Hospital (SGH), the national referral center for SJS/TEN.
Outcomes of interest included mortality, the incidence of bacteremia at SGH, ICU admission, and length of hospital stay.
Admissions occurring within 4 days of skin
blistering according to clinical records were considered early admissions and delayed admissions were defined as admissions occurring 5 days or more after skin blistering.
The consensus of the 5-day cut-off was
based on
1) a multi-center study in Europe, 1
wherein the median admission delay was 5-7 days across several centers and
2) the natural history of SJS/TEN, where the disease evolves from initial blistering to hospitalization in 3 days, then progresses to maximum detachment in another 5 days.
3) A total of 123 patients with SJS/TEN were included in this study.
There were 93 (76%) patients with early
admissions and 30 (24%) with delayed admissions.
Baseline characteristics are shown in Table I.
Univariate analyses demonstrated a significant difference in outcomes between early and late admission patients for mortality (14% vs 37%; P ¼ .007),
incidence of bacteremia (20% vs 52%; P ¼.001), rate of ICU admission (14% vs 41%; P ¼.002), and total length of stay at SGH (16 6 22 vs 28 6 23; P ¼ .01).
A dose response relationship was demonstrated between
mortality and admission delay, with mortality increasing from 15% in those admitted within 03 days, to 19% in those admitted within 4-5 days, and to 33% in those admitted within 6 days
Stevens-Johnson syndrome and toxic epidermal necrolysis are acute, rare, and potentially fatal skin reactions involving loss of skin and, in some cases, mucosal membranes accompanied by systemic symptoms.
Medications are causative in over 80 percent of cases. Stevens-Johnson syndrome and toxic epidermal necrolysis are distinguished based
Stevens-Johnson syndrome and toxic epidermal necrolysis are acute, rare, and potentially fatal skin reactions involving loss of skin and, in some cases, mucosal membranes accompanied by systemic symptoms.
Medications are causative in over 80 percent of cases. Stevens-Johnson syndrome and toxic epidermal necrolysis are distinguished based on the extent of the detached skin surface area.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are adverse reactions that affect the
mucocutaneous surfaces by causing necrosis and detachment of the epidermis. The difference between SJS
and TEN is in the percentage of the body surface area (BSA) affected. TEN is known to affect greater BSA
than SJS. The pathogenesis of SJS/TEN is attributed to drug-specific cell-mediated cytotoxic reactions that
directly and indirectly lead to keratinocyte apoptosis through mediators. Clinical presentation begins with
influenza-like symptoms, with the disease affecting the skin, oral, ocular, and urogenital regions most
frequently. Although SJS/TEN is mainly due to various drugs, infection and vaccination can also induce
SJS/TEN. This review outlines a compilation of all drugs implicated in SJS/TEN cases based on studies,
mainly in case reports and other study types. Drug classes implicated in SJS/TEN cases include antibiotics,
anticonvulsants, antineoplastics, analgesics, and diuretics, among others. There is no fully established
diagnostic modality for SJS/TEN; treatment is done mainly by withdrawing the offending agent. In addition
to withdrawing the offending agent, a multidisciplinary care team is essential in managing these patients.
Several pharmacologic modalities have also been proposed in treatment, but there is still insufficient
evidence for the efficacy of one against the other
The drugs that most commonly cause Stevens-Johnson syndrome/toxic epidermal necrolysis are:
The drugs that most commonly cause Stevens-Johnson syndrome/toxic epidermal necrolysis are:
Curr Opin Allergy Clin Immunol. 2024 Aug; 24(4): 218–227. Published online 2024 May 17. doi: 10.1097/ACI.0000000000000993
PMCID: PMC11213502
Purpose of review
Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) are severe cutaneous adverse drug
reactions (SCARs) characterized by widespread epithelial detachment and blistering, which affects the skin
and mucocutaneous membranes. To date, therapeutic interventions for SJS/TEN have focused on systematic
suppression of the inflammatory response using high-dose corticosteroids or intravenous immunoglobulin G
(IgG), for example. No targeted therapies for SJS/TEN currently exist.
Recent findings
Though our understanding of the pathogenesis of SJS/TEN has advanced from both an immunological and
dermatological perspective, this knowledge is yet to translate into the development of new targeted therapies.
Summary
Greater mechanistic insight into SJS/TEN would potentially unlock new opportunities for identifying or
repurposing targeted therapies to limit or even prevent epidermal injury and blistering.
Keywords
drug hypersensitivity, drug repurposing, pathogenesis, Stevens–Johnson syndrome, toxic epidermal necrolysis
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