Supportive care in the acute phase of Stevens–Johnson syndrome and toxic epidermal necrolysis: an international, multidisciplinary Delphi‐based consensus

Abstract

Background Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking. Objectives Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN. Methods Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method. Results Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements. Conclusions We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.What is already known about this topic?• Supportive care is the cornerstone of management of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in the acute phase.• There is no consensus or guideline on the best supportive care.

Toxic epidermal necrosis associated with afatinib: A case report and literature review

“…The typical latency period from drug exposure to symptoms ranges from 4 to 28 days ( 8 ). The incidence of SJS/TEN is 1-6 per 1,000,000, with a mortality rate of approximately 15% ( 9 ), most commonly resulting from sepsis ( 10 ). The histopathology of lesions in SJS/TEN varies from keratinocyte apoptosis to epidermal necrosis, with a partial infiltration of perivascular lymphocytes, histiocytes, and a few eosinophils being observed in the dermis ( 7 ).…”

Valproic acid induced aplastic crisis and Stevens-Johnson syndrome in a single pediatric patient

“…An international multidisciplinary consensus on supportive care in the acute phase of SJS and toxic epidermal necrolysis was published in 2021. It described 14 aspects of care, including immediate discontinuation of suspected drugs, prevention of infection, fluid resuscitation, nutritional support, local skin care, surveillance, and other measures [ 14 ].…”

Stevens–Johnson syndrome in children

“…Early transfer to an experienced center is recommended in severe cases [15 ▪▪ ]. Excellent detailed references on consensus-generated multidisciplinary supportive care for blistering SCARs [78 ▪ ] and care of children and adolescents with SJS-TEN [15 ▪▪ ] were recently published.…”