Toxic epidermal necrolysis: Review of pathogenesis and management

Downey, Andrew; Jackson, Chris; Harun, Nadia; Cooper, Alan

doi:10.1016/j.jaad.2011.09.029

Cited by 142 publications

(181 citation statements)

References 87 publications

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“…Compared with other methods of treatment, meticulous supportive therapy is the sole intervention that consistently improves prognosis. 7 Interestingly, results from the most The decision to use TPE in this patient was timely; the exchange halted the progression of his condition. The patient had a good response to treatment, with signs of improvement after the third cycle of the first series of plasmapheresis and IVIg.…”

Section: Painmentioning

confidence: 93%

“…Currently Fas-mediated pathways, granulysin, and reactive oxygen species are being investigated as possible early steps in TEN. 7 The clinical picture may begin with unspecific signs and symptoms such as fever, cough, rhinorrhea, or malaise followed by the appearance of unevenly shaped, macular, erythematous lesions on the face and trunk that spread to the extremities and later coalesce. Massive epidermal detachment ensues over the next 5 to 7 days, and reepithelialization occurs during the following weeks.…”

Section: Pathophysiology and Clinical Manifestationsmentioning

confidence: 99%

“…8 The results of our own experience 11 and the findings in other case reports, 12 case series, 13 and retrospective cohort studies 8 have consistently indicated clinical benefits with plasmapheresis, and additional research on this method has been recommended. 1,7 Regardless of the therapeutic option chosen, a comprehensive team approach that emphasizes supportive care is essential for any patient with TEN. Detachment of the epidermal layer results in marked fluid and electrolyte depletion, and loss of the skin's natural barrier properties increases the risk for infection and subsequent sepsis.…”

Section: Treatmentmentioning

confidence: 99%

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Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

Seczyńska

Nowak²,

Sega³

et al. 2013

Critical Care Nurse

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L yell disease, commonly referred to as toxic epidermal necrolysis (TEN), is a rare, lifethreatening condition probably related to drug hypersensitivity; the mortality rate is approximately 30%.1 The disease is characterized by an initial pruritic, erythematous rash followed by the appearance of bullae, which subsequently lead to ulceration and sloughing of the underlying epidermal base similar to physiological damages caused by burns. Moreover, TEN causes crusting, desquamation, and ulceration of the mucous membranes in areas such as the oral, ocular, and genital mucosa.2 Bleeding from internal mucous membranes, especially those in the gastrointestinal and respiratory tracts, can progress to hypovolemia and lead to death. TEN is often accompanied by ocular manifestations ranging from acute conjunctivitis to corneal abrasion and, in the most severe cases, corneal ulceration.3 These changes can lead to permanent loss of vision. Feature A patient with severe toxic epidermal necrolysis underwent 2 cycles of therapeutic plasma exchange and received specialized wound care for widespread skin damage of more than 80% of his body surface area. Extensive involvement of mucous membranes, including the conjunctivas and the oropharyngeal cavity, and damage of his genitourinary organs required meticulous wound care. Daily care of injuries of tissues affected only in the most severe cases of toxic epidermal necrolysis was provided by an experienced intensive care unit nursing team. A meticulous supportive therapy regimen was a major contributing factor to this patient's remission. Supportive Therapy(Critical Care Nurse.

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Section: Painmentioning

confidence: 93%

Section: Pathophysiology and Clinical Manifestationsmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

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Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

Seczyńska

Nowak²,

Sega³

et al. 2013

Critical Care Nurse

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“…Thus, from this study, the association between systemic corticosteroid use and the increased risk of gastrointestinal problems and infection emerged. This use also led to increased mortality, prompting the author of this study to suggest that the use of steroids for the treatment of TEN should be contraindicated 9,10 . IVIg may have contributed to this decline because it was reported relatively soon thereafter, in 1998, to have a plausible mechanism for the treatment of TEN 11 .…”

Section: Systemic Corticosteroidsmentioning

confidence: 99%

“…Although there have been no randomized controlled trials done, cyclosporine tends to be beneficial for the treatment of TEN (Table 3) 24 27 . The doses (3 10 mg/kg/day) and durations vary but have been reported to arrest the disease progression and promote re-epithelialization 9,12 . A limitation in the active use for the treatment of TEN is that the full therapeutic effect takes more than 1 month.…”

Section: Cyclosporinementioning

confidence: 99%

A Review of the Active Treatments for Toxic Epidermal Necrolysis

Kinoshita

Saeki

2017

J Nippon Med Sch

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Toxic epidermal necrolysis (TEN) is a severe adverse drug reaction associated with the separation of skin and mucous membranes at the dermal-epidermal junction. Although it is rare, many treatments have been trialed because of its high mortality rate. Active interventions performed to date include the use of systemic corticosteroids, intravenous immunoglobulins (IVIg), cyclosporine, plasmapheresis, antitumor necrosis factor drugs and N-acetylcysteine, but none has been established as the most effective therapy. IVIg and short-term high-dose corticosteroids were regarded as the most promising treatments for TEN in a comprehensive review of all reported TEN cases from 1975 2003. When used with an appropriate dose and timing, the beneficial effects of IVIg can be maximized. Although no randomized controlled trials have been conducted, cyclosporine and plasmapheresis are considered to be beneficial.As no gold standard for active intervention for TEN has been established, the choice of treatment relies partly on the available guidelines and the experience of the dermatologist. There is still much to be investigated regarding the pathogenesis of TEN, and new findings may contribute to the identification of an effective active intervention strategy. (J Nippon Med Sch 2017; 84: 110 117)

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Evaluation of Plasmapheresis vs Immunoglobulin as First Treatment After Ineffective Systemic Corticosteroid Therapy for Patients With Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

et al. 2023

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ImportanceStevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are severe cutaneous adverse reactions, and patients with SJS/TEN frequently require intensive care. However, there is limited evidence on the clinical outcomes of immunomodulating therapy, including plasmapheresis and intravenous immunoglobulin (IVIG) in patients with SJS/TEN.ObjectiveTo compare clinical outcomes of patients with SJS/TEN who were treated with plasmapheresis first vs IVIG first after ineffective systemic corticosteroid therapy.Design, Setting, and ParticipantsThis retrospective cohort study used data from a national administrative claims database in Japan that included more than 1200 hospitals and was conducted from July 2010 to March 2019. Inpatients with SJS/TEN who received plasmapheresis and/or IVIG therapy after initiation of at least 1000 mg/d of methylprednisolone equivalent systemic corticosteroid therapy within 3 days of hospitalization were included. Data were analyzed from October 2020 to May 2021.ExposuresPatients who received IVIG or plasmapheresis therapy within 5 days after initiation of systemic corticosteroid therapy were included in the IVIG- and plasmapheresis-first groups, respectively.Main Outcomes and MeasuresIn-hospital mortality, length of hospital stay, and medical costs.ResultsOf 1215 patients with SJS/TEN who had received at least 1000 mg/d of methylprednisolone equivalent within 3 days of hospitalization, 53 and 213 patients (mean [SD] age, 56.7 [20.2] years; 152 [57.1%] women) were included in the plasmapheresis- and IVIG-first groups, respectively. Propensity-score overlap weighting showed no significant difference in inpatient mortality rates between the plasmapheresis- and IVIG-first groups (18.3% vs 19.5%; odds ratio, 0.93; 95% CI, 0.38–2.23; P = .86). Compared with the IVIG-first group, the plasmapheresis-first group had a longer hospital stay (45.3 vs 32.8 days; difference, 12.5 days; 95% CI, 0.4–24.5 d; P = .04) and higher medical costs (US $34 262 vs $23 054; difference, US $11 207; 95% CI, $2789–$19 626; P = .009).Conclusions and RelevanceThis nationwide retrospective cohort study found no significant benefit to administering plasmapheresis therapy first instead of IVIG first after ineffective systemic corticosteroid treatment in patients with SJS/TEN. However, medical costs and length of hospital stay were greater for the plasmapheresis-first group.

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Toxic epidermal necrolysis: Review of pathogenesis and management

Cited by 142 publications

References 87 publications

Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

A Review of the Active Treatments for Toxic Epidermal Necrolysis

Evaluation of Plasmapheresis vs Immunoglobulin as First Treatment After Ineffective Systemic Corticosteroid Therapy for Patients With Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

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