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      Stevens-Johnson Syndrome

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          Abstract

          DESCRIPTION A 45-year-old African American HIV-positive man presented with a 6-day history of worsening rash that began on his trunk and progressed to involve his extremities, oral mucosa, and urethral meatus. He reported a recent history of starting sulfamethoxazole-trimethoprim for pneumocystis prophylaxis. QUESTIONS What is Stevens-Johnson syndrome (SJS) and how is it classified? What causes SJS and how does it present? How is SJS treated? What is the prognosis of SJS? DISCUSSION Stevens-Johnson syndrome is a severe and potentially lethal disease due to an immune-complex–mediated hypersensitivity reaction involving the mucous membranes and skin.1 First described in 1922, SJS was originally thought to be a variant of erythema multiforme (EM); however, this has fallen out of favor, as SJS is mainly due to a drug reaction whereas EM is mainly a parainfectious process.2 SJS occurs on a spectrum based upon total body surface area (TBSA) of skin affected, with the most severe form referred to as toxic epidermal necrolysis (TEN), or Lyell's syndrome. SJS encompasses less than 10% TBSA; SJS/TEN overlap occurs between 10% and 30% TBSA, and TEN encompasses more than 30% TBSA.2 SJS/TEN can occur in any individual but are more common in children and the elderly.3 Although there have been cases reported after a viral illness (mainly herpes simplex) or mycoplasma infections, more than 90% of cases are medication induced—the main culprits being antibiotics (sulfonamides and β-lactams), nonsteroidal anti-inflammatory drugs, and antiepileptics (phenytoin and carbamazepine).3,4 Initial presentation can be nonspecific, with fever, malaise, cough, sore throat, or eye discomfort all appearing before the cutaneous manifestations. Following this prodrome is the onset of mucosal ulcerations and then progression to cutaneous, dusky red vesiculobullous-appearing atypical target-like lesions on the trunk and face that evolve over the course of 2 to 15 days (Figs 1-3).3,5 These cutaneous manifestations have a positive Nikolsky sign due to dermal-epidermal junction involvement (Fig 2).6 If skin lesions are seen, biopsy is warranted to assist with diagnostic workup. The classic pathology findings are dermal monocyte infiltrate with full-thickness epidermal necrosis.1,3 Eye involvement can range from erythema and eyelid edema to corneal ulcerations and can be debilitating in the long term.1,3,5 The initial step in management should be discontinuation of medication suspected of precipitating the event—otherwise care is largely supportive. Patency of the airway, hemodynamic stability, and overall fluid status must continually be assessed. There should be a low threshold for enteral feeding tubes to provide the necessary calories for wound healing if oral ulcerations prevent adequate intake.1 Urinary catheters may be useful if genital involvement is present. In regard to cutaneous involvement, care is directed toward preventing shear forces that would further denude skin. Nonadherent silver-containing dressings can be used to cover all affected areas and minimize bacterial colonization of skin as well as reducing the amount of dressing changes that may further contribute to skin sloughing.7 Systemic therapy such as corticosteroids, intravenous immunoglobulin, prophylactic antimicrobials, and plasmapheresis is controversial, with no controlled study showing a clear benefit on mortality or reducing disease progression.3 Involved skin should not be excised or debrided. Consultations with subspecialists such a Critical Care, Ophthalmology, Urology, and Infectious Diseases should be considered. Disease prognosis is variable, with the risk of mortality increasing with the severity of the syndrome. SJS has a mortality of approximately 5% and TEN has the worst mortality of approximately 16% to 55%.3,6,8 Scoring systems, such as the SCORTEN, exist to assess the risk of mortality, with factors including patient age, TBSA involved, concomitant malignancy, as well as several initial serum chemistries.8 Immune-complex–mediated hypersensitivity reactions such as SJS/TEN can be devastating. Initial care should include discontinuation of the offending medication. Further management is supportive and aims to prevent secondary complications, hypovolemia, and infection. Wound care is provided with silver-containing dressings and preventing further shearing of skin. Patients should be monitored closely in an intensive care setting, with high suspicion for development of pulmonary or infectious processes, as these are the overwhelming cause of mortality.

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          SCORTEN: a severity-of-illness score for toxic epidermal necrolysis.

          The mortality of toxic epidermal necrolysis is about 30%. Our purpose was to develop and validate a specific severity-of-illness score for cases of toxic epidermal necrolysis admitted to a specialized unit and to compare it with the Simplified Acute Physiology Score and a burn scoring system. A sample of 165 patients was used to develop the toxic epidermal necrolysis-specific severity-of-illness score and evaluate the other scores, a sample of 75 for validation. Model development used logistic regression equations that were translated into probability of hospital mortality; validation used measures of calibration and discrimination. We identified seven independent risk factors for death and constituted the toxic epidermal necrolysis-specific severity-of-illness score: age above 40 y, malignancy, tachycardia above 120 per min, initial percentage of epidermal detachment above 10%, serum urea above 10 mmol per liter, serum glucose above 14 mmol per liter, and bicarbonate below 20 mmol per liter. For each toxic epidermal necrolysis-specific severity-of-illness score point the odds ratio was 3.45 (confidence interval 2.26-5.25). Probability of death was: P(death) = elogit/1 + elogit with logit = -4.448 + 1.237 (toxic epidermal nec-rolysis-specific severity-of-illness score). Calibration demonstrated excellent agreement between expected (19. 6%) and actual (20%) mortality; discrimination was also excellent with a receiver operating characteristic area of 82%. The Simplified Acute Physiology Score and the burn score were also associated with mortality. The discriminatory powers were poorer (receiver operating characteristic area: 72 and 75%) and calibration of the Simplified Acute Physiology Score indicated a poor agreement between expected (9.1%) and actual (26.7%) mortality. This study demonstrates that the risk of death of toxic epidermal necrolysis patients can be accurately predicted by the toxic epidermal necrolysis-specific severity-of-illness score. The Simplified Acute Physiology Score and burn score appear to be less adequate.
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            Toxic epidermal necrolysis and Stevens Johnson syndrome: our current understanding.

            Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN, Lyell's syndrome) are now considered to be distinct clinical entities within a spectrum of adverse cutaneous drug reactions of increasing severity based on their surface of skin detachment. Within this spectrum, SJS which can be considered as a minor form of TEN is characterized by less than 10% body surface area of skin detachment, and an average reported mortality of 1-5%, whereas TEN is characterized by more than 30% skin detachment, and an average reported mortality 25-35%. Both SJS and TEN are characterized morphologically by the rapid onset of keratinocyte cell death by apoptosis, a process that results in the separation of the epidermis from the dermis. Recent evidence is supportive of a role for inflammatory cytokines and the death receptor Fas and its ligand FasL in the pathogenesis of keratinocyte apoptosis during TEN. This Fas-mediated keratinocyte apoptosis that is the last step culminating in epidermal detachment in TEN can be inhibited in vitro by antagonistic monoclonal antibodies to Fas, and by intravenous immunoglobulins (IVIG) which have been shown to contain natural anti-Fas antibodies. Consequently, over the last few years, numerous case reports and 9 non-controlled clinical studies containing 10 or more patients have analyzed the therapeutic effect of IVIG in TEN. Taken together, although each study has its potential biases, 7 of 9 such studies point towards a benefit of IVIG used at doses greater than 2 g/kg on the mortality associated with TEN. These studies should serve as the basis for designing an appropriate prospective trial or for conducting a metaanalysis in the near future, in order to determine the therapeutic efficacy of IVIG in TEN.
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              Antishear therapy for toxic epidermal necrolysis: an alternative treatment approach.

              Toxic epidermal necrolysis syndrome is a devastating disease, with mortality rates ranging between 20 and 60 percent. This study evaluated an alternative treatment approach using antishear wound care and compared outcomes using the severity of illness score for toxic epidermal necrolysis syndrome (SCORTEN) system. Records of 48 patients with a histopathologic diagnosis of toxic epidermal necrolysis syndrome treated with burn equivalent critical care and antishear wound care from September of 1985 to April of 2004 were reviewed. Observed mortality data were compared with those expected using the SCORTEN, and the standardized mortality ratio was calculated. The overall mortality rate was 27 percent. Factors affecting mortality were advancing age, time to burn unit admission, multisystem organ failure, and presence of comorbidities (p = 0.02, p = 0.02, p < 0.001, and p = 0.003, respectively). Chronic renal insufficiency and malignancy were two independent risk factors for nonsurvival (p = 0.04 and p = 0.004, respectively). Patients with a SCORTEN score of 2 or less had no mortality rate in this series. Observed and predicted mortality rates were comparable for patients with SCORTEN scores of 3 or greater. Patients with combined scores of 3 or less had a standardized mortality ratio score of 0.58 (42 percent mortality reduction). Overall, the standardized mortality ratio was 0.89 (11 percent mortality reduction). Transfer to a burn intensive care unit and initiation of critical care and wound protocols similar to those used for burn patients are recommended for patients with toxic epidermal necrolysis syndrome. Antishear wound care provides an effective alternative wound care approach with equivalent mortality rates.
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                Author and article information

                Journal
                Eplasty
                Eplasty
                ePlasty
                Eplasty
                Open Science Company, LLC
                1937-5719
                2016
                7 December 2016
                : 16
                : ic47
                Affiliations
                [1] aDivision of Plastic Surgery, Department of Surgery, University of South Florida Morsani College of Medicine, Tampa
                [2] bDepartment of Emergency Medicine, Florida Hospital, Orlando
                Author notes
                Article
                47
                5155313
                85d6ee18-e934-47a8-99d8-1214d4e7fc3e
                Copyright © 2016 The Author(s)

                This is an open-access article whereby the authors retain copyright of the work. The article is distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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                Surgery
                stevens-johnson syndrome,sjs,toxic epidermal necroysis,ten,rash
                Surgery
                stevens-johnson syndrome, sjs, toxic epidermal necroysis, ten, rash

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