Multidisciplinary Treatment in Toxic Epidermal Necrolysis

Abstract

Toxic epidermal necrolysis, Leyll’s syndrome (TEN), is a rare mucocutaneous blistering disease burdened with high mortality rates. The diagnosis of TEN is based on clinical symptoms and histopathological findings. In approximately 90% of cases, it is a severe adverse reaction to drugs. In TEN, not only is the skin affected, but also mucosa and organs’ epithelium. There are no unequivocal recommendations in regard to systemic and topical treatment of the patients. The aim of this paper is to review available literature and propose unified protocols to be discussed. Early management and multidisciplinary treatment are necessary to improve patients’ outcome. Treatment of patients with TEN suspicions should be initiated with early drug withdrawal. TEN patients, like patients with burns, require intensive care and multidisciplinary management. Each patient with TEN should be provided with adequate fluid resuscitation, respiratory support, nutritional treatment, pain control, infection prophylaxis, anticoagulant therapy, and gastric ulcer prophylaxis. The key to local treatment of patients with TEN is the use of nonadherent dressings that do not damage the epidermis during the change. The aim of the systemic treatment is purification of the blood stream from the causative agent. The most efficient way to clarify serum of TEN patients’ is the combination of plasmapheresis and IVIG. Immunomodulatory therapy can reduce the mortality five times in comparison with the patients with immunosuppression or lack of full protocol.

Pain Management in Dermatology

“…Adding an anxiolytic to local and general analgesic treatments can improve anxiety and the perception of acute pain [29]. The use of non-adherent dressings is recommended [27], and the Society of Dermatology Hospitalists pleads for preserve detached epidermis as a biologic dressing [30].…”

“…The management recommendations are similar to those for burn patients [27], as those patients are indeed often admitted to the burn unit [28]. Frequent pain assessment is necessary [27, 29].…”

“…The management recommendations are similar to those for burn patients [27], as those patients are indeed often admitted to the burn unit [28]. Frequent pain assessment is necessary [27, 29]. Recommendations for supportive care have been proposed by the Society of Dermatology Hospitalists in 2020 [30].…”

Tata Laksana Terintegrasi Sindrom Stevens-Johnson (SJS) dan Nekrolisis Epidermal Toksik (NET)

“…2 Keterlibatan multiorgan dapat terjadi pada SSJ-NET, sehingga tata laksana multidisiplin diperlukan untuk terapi yang optimal. 20 Sejumlah 90% pasien dalam 1 tahun dapat mengalami gejala sisa kulit (kering, pigmentasi, rambut, kuku, dan abnormalitas berkeringat), mata (dry-eye syndrome, konjungtivitis sikatriks, memendeknya forniks konjungtiva, simblefaron, abnormalitas kornea, gangguan fungsi visual), mulut (kering, perubahan gigi, pertumbuhan abnormal pada anak), genital, dan dapat juga mengenai saluran cerna dan bronkus. Sering terjadi post-traumatic stress disorder (PTSD).…”

Etanercept Combined with Glucocorticoid and Gamma Globulin for Treating Children with Toxic Epidermal Necrolysis: A Case Report

“…Clinically, conventional treatments such as cyclophosphamide, cyclosporine and glucocorticoid (GC) therapy are often employed but are accompanied by significant side effects and an increased risk of infection. 2,3 Although previous literature has discussed using biological agents in treating adult patients with TEN, scant information exists regarding paediatric patients. 4 To date, only 4 children with toxic epidermal necrolysis have been treated with infliximab and 2 with etanercept.…”