Beneficial effect of plasma exchange in the treatment of toxic epidermal necrolysis: A series of four cases

Košťál, Milan; Bláha, M.; Lánska, M.; Košt́álová, Marie; Bláha, V.; Štepánová, Eva; Malý, J

doi:10.1002/jca.21213

Cited by 34 publications

(20 citation statements)

References 23 publications

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“…Plasmapheresis is valued for its rapid results, rare side effects, and efficiency in treating patients with TEN refractory to steroids or IVIg. 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.…”

Section: Treatmentmentioning

confidence: 80%

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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: Treatmentmentioning

confidence: 80%

“…These results are similar to outcomes in previous reports of therapeutic success in severe, refractory cases of TEN treated with plasmapheresis. [10][11][12][13] The reason for the outcome is unclear. Researchers speculate that plasmapheresis removes necrosis-inducing factors from the bloodstream, but these factors have yet to be identified.…”

Section: Painmentioning

confidence: 99%

Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

Seczyńska

Nowak²,

Sega³

et al. 2013

Critical Care Nurse

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“…2 Similarly, elevated levels of Fas-L have been found in TEN patients, 16 and a case series showed resolution of the TEN mucosal damage once plasmapheresis was initiated, correlating with removal of Fas-L from the serum. 17 Its use in patients with isolated VBDS is limited. A case report showed minimal improvement after plasma exchange.…”

Section: Discussionmentioning

confidence: 99%

Infliximab/Plasmapheresis in Vanishing Bile Duct Syndrome Secondary to Toxic Epidermal Necrolysis

White¹,

Appleman²

2014

Pediatrics

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Vanishing bile duct syndrome (VBDS) is a rare disorder characterized by loss of interlobular bile ducts and progressive worsening cholestasis. The acute presentation of this disease is typically associated with a drug hypersensitivity and Stevens-Johnson syndrome/toxic epidermal necrolysis (TEN). The mainstay of treatment has been ursodeoxycholic acid with mixed results from immunosuppressive regimens. Anti–tumor necrosis factor-α and plasmapheresis have been speculated to be of potential benefit. It is hoped that early identification and intervention in VBDS secondary to Stevens-Johnson syndrome/TEN with continued reporting will lead to better regimens and outcomes. Our case report details the first reported use of infliximab and plasmapheresis, in addition to steroids, in a patient with VBDS secondary to TEN, as well as a literature review that supports a mechanism for why these modalities could be effective treatments. Unfortunately, our patient died, and the use of these therapies had an unclear benefit on his liver and skin disease. We hope that additional work can be published to confirm or refute their utility in the treatment of these diseases.

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“…It should be considered only once the patient is stable and if the skin damage is still ongoing and doesn’t respond to other conventional therapies (corticosteroids or IVIG). Plasmapheresis may have a role in the treatment of ED because it removes Fas-L [96], other cytokines known to be implied in the pathogenesis (IL-6, IL-8, TNF-α) [97, 98]. Moreover Mawson A and colleagues hypothesized that the efficacy of plasmapheresis is able to reduce serum level of vitamin A.…”

Section: Management and Therapymentioning

confidence: 99%

Drug induced exfoliative dermatitis: state of the art

et al. 2016

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Drug induced exfoliative dermatitis (ED) are a group of rare and severe drug hypersensitivity reactions (DHR) involving skin and usually occurring from days to several weeks after drug exposure. Erythema multiforme (EM), Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the main clinical presentations of drug induced ED. Overall, T cells are the central player of these immune-mediated drug reactions. Here we provide a systematic review on frequency, risk factors, pathogenesis, clinical features and management of patients with drug induced ED.Electronic supplementary materialThe online version of this article (doi:10.1186/s12948-016-0045-0) contains supplementary material, which is available to authorized users.

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Beneficial effect of plasma exchange in the treatment of toxic epidermal necrolysis: A series of four cases

Abstract: Plasmapheresis should be considered as an alternative treatment modality for patients with the most severe form of TEN if initial treatment with other agents, including corticosteroids and/or IVIG, fails. Drugs were suspected to be the cause of TEN in all four cases.

Cited by 34 publications

References 23 publications

Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

Supportive Therapy for a Patient With Toxic Epidermal Necrolysis Undergoing Plasmapheresis

Infliximab/Plasmapheresis in Vanishing Bile Duct Syndrome Secondary to Toxic Epidermal Necrolysis

Drug induced exfoliative dermatitis: state of the art

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