Updates in SJS/TEN: collaboration, innovation, and community

Abstract

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) is a predominantly drug-induced disease, with a mortality rate of 15–20%, that engages the expertise of multiple disciplines: dermatology, allergy, immunology, clinical pharmacology, burn surgery, ophthalmology, urogynecology, and psychiatry. SJS/TEN has an incidence of 1–5/million persons per year in the United States, with even higher rates globally. One of the challenges of SJS/TEN has been developing the research infrastructure and coordination to answer questions capable of transforming clinical care and leading to improved patient outcomes. SJS/TEN 2021, the third research meeting of its kind, was held as a virtual meeting on August 28–29, 2021. The meeting brought together 428 international scientists, in addition to a community of 140 SJS/TEN survivors and family members. The goal of the meeting was to brainstorm strategies to support the continued growth of an international SJS/TEN research network, bridging science and the community. The community workshop section of the meeting focused on eight primary themes: mental health, eye care, SJS/TEN in children, non-drug induced SJS/TEN, long-term health complications, new advances in mechanisms and basic science, managing long-term scarring, considerations for skin of color, and COVID-19 vaccines. The meeting featured several important updates and identified areas of unmet research and clinical need that will be highlighted in this white paper.

Recent findings on drug hypersensitivity in children

“…SJS and TEN are rare severe cutaneous adverse drug reactions mediated by cytotoxic T cells (mainly CD8+). There is extensive epidermal necrosis and detachment, with mucocutaneous complications ( 49 , 50 ). SJS and TEN differ only along a spectrum of severity based on percentage of body surface area detached (<10% in SJS; 10%–30% in an overlap SJS/TEN; and >30% in TEN) ( 49 ).…”

“…Hsu et al reported an incidence rate of 5.3 and 0.4 cases per million children for SJS and TEN, respectively, with an equal incidence between male and female children ( 51 ). In children, as in adults, drugs are the most frequent triggers of SJS/TEN ( 49 , 50 ). Common culprit medications are sulfa antibiotics, AED, and NSAIDs ( 49 ).…”

“…The early manifestations of SJS/TEN include fever, malaise, and anorexia ( 50 ). It progresses with cutaneous lesions and bullae.…”

“…It progresses with cutaneous lesions and bullae. SJS/TEN can mimic other illnesses, such as erythema multiforme major (EMM), staphylococcal scalded skin syndrome (SSSS), or autoimmune blistering diseases ( 49 , 50 ). Table 2 shows some differences in clinical characteristics and main causes between SJS/TEN and other cutaneous diseases ( 49 – 56 ).…”

“…The treatment is based on early detection and removal of the offending agents. Patients should be admitted to an intensive care unit or burn center as quickly as possible ( 49 , 50 ). Supportive care is comprised of wound and eye care; fluid and electrolyte management; nutritional support; and prompt treatment of infections ( 49 , 50 ).…”