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402
W. Y. Rockne et al.
26. Mozingo DW. The pentagon attack of September 11, 2001: a
burn center’s experience. Yearbook of Surgery 2006;2006:49–50.
doi:https://doi.org/10.1016/s0090- 3671(08)70336- 7.
2 7. Cushman JG, Pachter HL, Beaton HL. Two New York City
hospitals’ surgical response to the September 11, 2001, terrorist
attack in New York City. J Trauma. 2003;54(1):147–55. https://
doi.org/10.1097/00005373- 200301000- 00018.
28. Sheridan RL, Friedstat J, Votta K. Lessons learned from burn
disasters in the post-9/11 ERA.Clin Plast Surg. 2017;44(3):435–
40. https://doi.org/10.1016/j.cps.2017.02.003.
29. Yurt RW, Bessey PQ, Alden NE, et al. Burn-injured
patients in a disaster: September 11th revisited. J Burn
Care Res. 2006;27(5):635–41. https://doi.org/10.1097/01.
bcr.0000236836.46410.f2.
30. Matsunari Y, Yoshimoto N. Comparison of rescue and relief
activities within 72 hours of the atomic bombings in Hiroshima
and Nagasaki. Prehosp Disaster Med. 2013;28(6):536–42. https://
doi.org/10.1017/s1049023x13008832.
31. Goffman TE. Nuclear terrorism and the problem of burns.
Am J Emerg Med. 2011;29(2):224–8. https://doi.org/10.1016/j.
ajem.2009.03.022.
32. DiCarlo AL, Maher C, Hick JL, et al. Radiation injury after
a nuclear detonation: medical consequences and the need for
scarce resources allocation. Disaster Med Public Health Prep.
2011;5(S1):S32–44. https://doi.org/10.1001/dmp.2011.17.
33. Phua YS, Miller JD, Wong She RB. Total care requirements of
burn patients: implications for a disaster management plan.
J Burn Care Res. 2010;31(6):935–41. https://doi.org/10.1097/
bcr.0b013e3181f93938.
34. Sheridan R, Barillo D, Herndon D, etal. Burn specialty teams. J
Burn Care Rehabil. 2005;26(2):170–3. https://doi.org/10.1097/01.
bcr.0000155544.38709.6e.
35. Klein MB.Geographic access to burn center hospitals. JAMA.
2009;302(16):1774. https://doi.org/10.1001/jama.2009.1548.
36. Hick JL, Barbera JA, Kelen GD. Refining surge capacity:
conventional, contingency, and crisis capacity. Disaster Med
Public Health Prep. 2009;3(2 Suppl):S59–67. https://doi.
org/10.1097/dmp.0b013e31819f1ae2.
3 7. Kearns RD, Cairns CB, Holmes JH 4th, Rich PB, Cairns
BA. Thermal burn care: a review of best practices. What
should prehospital providers do for these patients? EMS World.
2013;42(1):43–51.
Chapter 18. Burn Disasters
Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/
403
38. Kearns RD, Bettencourt AP, Hickerson WL, et al. Actionable,
revised (v.3), and amplified American Burn Association triage
tables for mass casualties: a civilian defense guideline. J Burn
Care Res. 2020;41(4):770–9. https://doi.org/10.1093/jbcr/iraa050.
39. Abir M, Davis MM, Sankar P, Wong AC, Wang SC. Design of a
model to predict surge capacity bottlenecks for burn mass casualties at a large academic medical center. Prehosp Disaster Med.
2012;28(1):23–32. https://doi.org/10.1017/s1049023x12001513.
40. Conlon KM, Bell R, Lee RA, Marano M.Determining immediate
burn bed availability to support regional disaster response. J
Burn Care Res. 2019;40(6):832–7. https://doi.org/10.1093/jbcr/
irz094.
41. Sasser SM. In a moment’s notice: surge capacity for terrorist
bombings: challenges and proposed solutions. Atlanta: U.S.Dept.
of Health and Human Services. Centers for Disease Control and
Prevention, National Center for Injury Prevention and Control,
Division of Injury Response; 2007.
42. Wachtel TL, Cowan ML, Reardon JD. Developing
a regional and national burn disaster response. J
Burn Care Rehabil. 1989;10(6):561–7. https://doi.
org/10.1097/00004630- 198911000- 00021.
43. Who we are. American Burn Association. https://ameriburn.org/
who- we- are/. 2017. Accessed 1 Sept 2021.
44. Kelen GD, McCarthy ML.The science of surge. Acad Emerg Med.
2006;13(11):1089–94. https://doi.org/10.1197/j.aem.2006.07.016.
45. Verification criteria effective October 1, 2019. American Burn
Association. https://ameriburn.org/quality- care/verification/
verification- criteria/verification- criteria- effective- october1- 2019/. 2021. Accessed 1 Sept 2021.
46. Hick JL, Einav S, Hanfling D, Kissoon N, Dichter JR, Devereaux
AV, Christian MD, Task Force for Mass Critical Care; Task Force
for Mass Critical Care. Surge capacity principles: care of the
critically ill and injured during pandemics and disasters: CHEST
consensus statement. Chest. 2014;146(4 Suppl):e1S–e16S. https://
doi.org/10.1378/chest.14.
4 7. Harrington D, Holmes J, Conlon K, Jeng J. An update on
the regional organizations of the American Burn Association.
J Burn Care Res. 2014;35(3):228–32. https://doi.org/10.1097/
bcr.0b013e318295789f.
48. Presidential Policy Directive 8: national preparedness.
Department of Homeland Security. 2018. http://www.dhs.gov/
xabout/laws/gc_1215444247124.shtm. Accessed 1 Sept 2021.
404
W. Y. Rockne et al.
49. Office of the Assistant Secretary for preparedness and response;
2016. 2017–2022 health care preparedness and response
capabilities. https://www.phe.gov/Preparedness/planning/hpp/
reports/Documents/2017- 2022- healthcare- pr- capabilities.pdf.
Accessed 1 Sept 2021.
50. Strategic National Stockpile. phe.gov. https://www.phe.gov/
about/sns/Pages/default.aspx. Accessed 1 Sept 2021.
51. Kraft M, Marks E.U.S. government counterterrorism: a guide to
who does what. Boca Raton: CRC Press; 2012.
52. Hickerson WL, Ryan CM, Conlon KM, etal. What’s in a name?
Recent key projects of the committee on organization and
delivery of burn care. J Burn Care Res. 2015;36(6):619–25.
https://doi.org/10.1097/bcr.0000000000000189.
53. Saffle JR, Gibran N, Jordan M.Defining the ratio of outcomes
to resources for triage of burn patients in mass casualties. J Burn
Care Rehabil. 2005;26(6):478–82. https://doi.org/10.1097/01.
bcr.0000185452.92833.c0.
54. Taylor SR, Jeng JG, Saffle JL, Sen S, Greenhalgh D, Palmieri
T. Redefining the outcomes to resources ratio for burn patient
triage in a mass casualty. J Burn Care Res. 2014;35(1):41–5.
https://doi.org/10.1097/bcr.0000000000000034.
Chapter 19
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Exfoliative Skin Diseases:
Stevens-Johnson
Syndrome andToxic
Epidermal Necrolysis
FeliciaN.Williams andJongO.Lee
Introduction
Stevens-Johnson Syndrome (SJS), Toxic Epidermal Necrolysis
(TEN), and SJS/TEN overlap syndrome represent a spectrum of rare, but life-threatening type IV hypersensitive
reactions to medications or infections [1–4]. The characteristic pathologic finding is complete or full-thickness necrosis or
complete separation of the epidermis [1]. The reaction can
present on any and every mucosal surface, as well as the skin
[1]. The clinical diagnosis of SJS versus SJS/TEN overlap,
versus TEN is based upon the severity or amount of skin
desquamation. The loss of skin increases the risk of infection,
F. N. Williams (*)
Department of Surgery, University of North Carolina School of
Medicine, Chapel Hill, NC, USA
North Carolina Jaycee Burn Center, Chapel Hill, NC, USA
e-mail: fnwmd@med.unc.edu
J. O. Lee
Department of Surgery, University of Texas Medical Branch,
Galveston, TX, USA
Shriners Children’s Texas, Galveston, TX, USA
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2023
J. O. Lee (ed.), Essential Burn Care for Non-Burn Specialists,
https://doi.org/10.1007/978-3-031-28898-2_19
405
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F. N. Williams and J. O. Lee
sepsis, multiple organ dysfunction, and death, as skin is the
patients’ first line of defense against infection [5]. Mortality is
related to the amount of skin desquamation, patient risk factors, and comorbidities [6, 7]. Major complications, increased
morbidity and mortality related to SJS or TEN are similar in
nature to those experienced by patients with major burn injuries. Upon suspicion of any of these diagnoses, patients warrant prompt transfer and treatment in a burn center [2, 4].
Burn centers have multi-disciplinary teams of specialized
physicians, nurses, nutritionists, and rehabilitation personnel
trained to manage patients critically ill from skin loss, and the
subsequent metabolic and physiologic derangements [8]. This
chapter discusses the pathophysiology of SJS and TEN, and
current management guidelines.
Epidemiology
The incidence of SJS and TEN is approximately 1–10 cases
per million yearly, with SJS being the most prevalent—occurring three times more commonly [9, 10]. Hypersensitive reactions to medications are responsible for up to 80% of cases
[10]. Other causes are infectious (Mycoplasma Pneumonia or
Cytomegalovirus) or vaccines [10]. There are up to 20% of
cases without an identified cause [11]. It effects all ages, races,
ethnicities, and gender but has been reported to be more
common among older populations and more common in
women [9, 10]. Significant risk factors for development of SJS
and TEN include, but are not limited to having active malignancy, immune dysfunction or dysregulation, infection/sepsis,
epilepsy, renal or liver dysfunction, or a genetic predisposition
[4, 12]. Patients with a history of human immunodeficiency
virus (HIV) have a higher incidence [4, 10]. Using the
National Inpatient Sample from 2009 to 2012, which accounts
for 20% of all admissions in the United States, Hsu et al.
found that non-Hispanic white patients were least likely to
Chapter 19. Exfoliative Skin Diseases: Stevens-Johnson…
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present with SJS or TEN compared to all other races and
ethnicities [4]. Like other groups, patients were more likely to
be older and more likely to be women [4, 9, 10]. Patients were
also more likely to have multiple comorbidities [4]. Mortality
estimates are based upon degree of epidermal necrosis and
detachment and have ranged from 12 to 40% in previous
studies [13, 14]. Updated estimates from Hsu etal. show mortality risk for SJS at 4.8%, SJS/TEN overlap at 19.4% and
TEN at 14.8% [4].
407
Pathophysiology (Histopathology/
Morphology)
SJS and TEN are T-cell mediated reactions [10]. For druginduced reactions, natural killer cells and CD8+ T cells—
which may be drug specific—induce apoptosis of keratinocytes
and cause the activation and release of soluble cytotoxic
mediators in SJS and TEN) [12]. In the cases of SJS or TEN
secondary to viruses or autoimmune diseases, the factors contributing to widespread epidermal necrolysis are not completely understood [9]. Histopathological findings are
subepidermal vesicles or blisters with widespread epidermal
necrosis and apoptotic keratinocytes associated with mild or
minimal lymphocytic infiltration [9]. Epidermal necrolysis is
characterized by blister formation, partially or completely
detached skin, erythematous skin, and flat, atypical target
lesions. In almost all cases, mucous membranes are involved
[9]. There is a temporal transition of lymphocytic infiltration
in SJS and TEN.Early in the course of the syndrome, blister
fluid is mainly composed of cytotoxic CD8+ T cells and natural killer cells. The composition of lymphocytes transitions to
mainly monocytes later in the clinical course [9, 15]. Other
noted cell types of significance are granulysin, found in cytotoxic granules, Fas-FasL, perforin, granzyme B, TNF-alpha,
and nitrous oxide [10].
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F. N. Williams and J. O. Lee
Clinical Presentation
SJS was first described in the 1920s. Drs. Stevens and Johnson
reported on the mucocutaneous eruption in two children with
concurrent ocular involvement [16]. It can result from
Mycoplasma pneumoniae or Herpes Simplex virus infection
or from a severe drug reaction [17]. TEN was a term used by
Lyell in 1950s to describe a skin rash that appears similar to a
scald [18]. The most common etiology for TEN is drug
induced [17, 19]. Traditionally, patients are exposed to an incit-
ing agent. Between days to usually up to 4weeks, patients
may then experience flu-like symptoms (fevers, chills, malaise, sore throat, myalgias, cough, rhinitis, headache, or generalized pain in the skin, eyes, or mucosal surfaces) [10, 20]. If
systemic symptoms occur, they precede both mucosal and
skin involvement [1]. Patients typically present secondary to
the skin manifestations. They present with painful atypical
targetoid lesions or bullae, or red erythematous violaceous
patches or ulcers. When examined, or rubbed, the epidermal
bullae will detach from the dermis—positive Nikolsky sign
[1]. Up to 80% of patients have mucosal involvement—the
hallmark of SJS/TEN [1]. The oral cavity is more involved
than the eyes, genitals, or anus, but the entirety of the gastrointestinal and respiratory tracts may be involved [10, 11].
Acutely, patients are at risk for multi-system organ
dysfunction [4, 9–11]. Over 40% of patients are at risk for
pneumonia [9]. Nearly 15% of patients are at risk for renal
dysfunction [9]. Gastrointestinal and cardiovascular complications are also common [9]. The loss of skin increases the
risk of infection and sepsis as skin is the patients’ first line of
defense against infection [5]. Patients are in high risk of bacterial infections, with one single-center study reporting over
90% of patients with SJS or TEN with bacterial infections
and over 60% of patients with sepsis [9, 21]. Long-term complications are often related to the extent and duration of
symptoms during the acute phase. Patients may develop strictures in their gastrointestinal tract, genital adhesions, ocular
impairment, or bronchiolitis obliterans [9].
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409
Mortality is related to the amount of skin desquamation,
patient risk factors, and comorbid conditions [6, 7]. Hsu etal.
found that the main predictors of mortality were increasing
age, number of chronic conditions, infection, malignancy, and
renal failure [4]. There have been multiple scoring systems
developed to help prognosticate mortality risk for SJS and
TEN patients [7].
Management
Acute management of a patient suspected of having SJS or
TEN is supportive and requires cessation of the causative
agent, resuscitation, and evaluation or transfer to a burn center [9]. Major complications, increased morbidity and mortality related to SJS or TEN are similar in nature to those
experienced by patients with major burn injuries. Upon suspicion of any of these diagnoses, patients warrant prompt
transfer and treatment in a burn center [2, 4]. Burn centers
have multi-disciplinary teams of specialized physicians,
nurses, dietitians, and rehabilitation personnel trained to
manage patients critically ill from skin loss, and the subsequent metabolic and physiologic derangements from that loss
[8]. There has been an increased mortality in cases of delayed
admission of 7days or more after onset of symptoms to specialized centers [22].
One of the most critical steps in the evaluation of a
patient suspected of having SJS or TEN is the history and
physical examination. Identifying and stopping possible
causative agents are lifesaving. The class of drug, first and
last dose, half-life, and any potential previous exposures are
important information in the early phases and treatment of
patients with SJS and/TEN [9, 23]. Classes of drugs are
important to note. If the medication was related to seizures,
stopping may increase the patient’s risk of seizures. If the
possible causative agent is an antimicrobial, immediate cessation may increase the patient’s risk of infection or sepsis
which will increase their risk of mortality [4, 9]. Timing of
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F. N. Williams and J. O. Lee
medication may change the differential diagnosis [10]. In
addition, medications with a long half-life may be associated
with a higher mortality risk [23].
Clinical evaluation by dermatology is recommended.
While the diagnosis is clinical, many centers prefer fullthickness skin biopsy confirmation, which may determine
treatment options. In addition, determining the severity or
amount of epidermal detachment is important. The clinical
diagnosis of SJS versus SJS/TEN overlap, versus TEN is
based upon the severity or amount of skin desquamation.
Specifically, SJS involves up to 10% of (TBSA) epidermal
detachment. SJS/TEN overlap involves 10–30% TBSA epidermal detachment. TEN involves over 30% TBSA epidermal detachment [9, 10, 12, 17, 23–25]. To date, there remains a
lack of consensus about debridement [9, 26]. As in burns,
nutrition, preferably enteral is paramount to improve outcomes [26].
Beyond supportive measures, there are a number of
treatment options used to mitigate SJS and/or TEN.The ones
discussed here are corticosteroids, immunoglobulins (IVIG),
and cyclosporine. Corticosteroids use in SJS and TEN have
not been shown to have a mortality benefit compared to supportive care alone. There have been links to higher risks of
infections and sepsis [9, 26, 27].
IVIG inhibits Fas-mediated keratinocyte apoptosis [28].
Due to the suspected pathophysiology of SJS and TEN, IVIG
has been used with mixed results. While there is a trend
toward a mortality benefit, due to the heterogeneity of dosing
and patient populations, its use remains controversial [9].
Cyclosporine is a calcineurin inhibitor, thus an inhibitor of
cytotoxic T cells. It may have a potential therapy to help stop
disease progression and promote healing though it is contraindicated for patients with renal failure and/or immune
deficiency, or malignancy. There have been some reports with
mortality benefit but these studies must be evaluated with
caution due to the patient populations [9, 29, 30].
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411
Risks and benefits of the potential therapies beyond
supportive care should be weighed in a multi-disciplinary
setting due to increased risks of morbidity and mortality of
SJS and TEN and the potential therapies.
Conclusion
SJS and TEN, and SJS/TEN overlap syndrome represent a
spectrum of rare, but life-threatening type IV hypersensitive
reactions to medications or infections. The loss of skin
increases the risk of infection, sepsis, multi-system organ dysfunction, and death. Major complications related to SJS or
TEN are similar in nature to those experienced by patients
with major burn injuries. Upon suspicion of any of these diagnoses, patients warrant prompt transfer and treatment in a
burn center. Management is primarily supportive care, but
steroids, IVIG, and cyclosporine are immunomodulating
therapies that can be used for treatment. Most important
treatment options are to stop the causative agent, resuscitate,
and promptly transfer to a specialized center able to treat
SJS/TEN with multi-disciplinary specialists that can effectively treat the acute syndrome and mitigate long-term
consequences.
References
1. Schneider JA, Cohen PR.Stevens-Johnson syndrome and toxic
epidermal necrolysis: a concise review with a comprehensive
summary of therapeutic interventions emphasizing supportive
measures. Adv Ther. 2017;34:1235–44.
2. Richard EB, Hamer D, Musso MW, Short T, O’Neal HR Jr.
Variability in management of patients with SJS/TEN: a survey of
burn unit directors. J Burn Care Res. 2018;39:585–92.
3. Pichler WJ.Delayed drug hypersensitivity reactions. Ann Intern
Med. 2003;139:683–93.
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