From the Guidelines
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) require immediate discontinuation of the suspected triggering medication and transfer to a burn unit or intensive care setting, with supportive care as the mainstay of treatment, including fluid and electrolyte management, wound care, pain control, nutritional support, and prevention of infection, as recommended by the most recent guidelines 1.
Key Considerations
- SJS and TEN are severe, potentially life-threatening skin reactions characterized by widespread epidermal detachment and mucosal involvement, with a mortality rate ranging from 5-10% for SJS to 30-40% for TEN 1.
- Management requires a multidisciplinary team (MDT) approach, including specialists in dermatology, plastic surgery, intensive care, ophthalmology, and specialist skincare nursing, with additional input from other clinicians as needed 1.
- Supportive care is crucial, including fluid and electrolyte management, wound care, pain control, nutritional support, and prevention of infection, with the goal of reducing morbidity and mortality, and improving quality of life 1.
- Systemic corticosteroids, intravenous immunoglobulin (IVIG), or cyclosporine may be used in some cases, although their efficacy remains debated, and should be considered on a case-by-case basis, taking into account the individual patient's needs and circumstances 1.
Important Outcomes
- Survivorship and survival are critical outcomes in the management of SJS and TEN, with a focus on reducing mortality and improving quality of life 1.
- Internal organ dysfunction and support, as well as prevention of long-term morbidity, are also important considerations in the management of these conditions 1.
- Quality of life and psychosocial well-being are essential outcomes, with a focus on minimizing the impact of SJS and TEN on the patient's daily life and overall well-being 1.
From the Research
Overview of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
- Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe dermatologic conditions with a high mortality rate, ranging from 15% to 25% 2.
- These conditions are characterized by mucous membrane and epidermal detachment, and are often triggered by medications, particularly antimicrobials, antiepileptics, allopurinol, and nonsteroidal anti-inflammatory medications 2.
Diagnosis and Management
- Diagnosis of SJS/TEN is primarily clinical, and appropriate therapy should be instituted without delay 2.
- Management of SJS/TEN requires a multidisciplinary team of physicians experienced in the care of patients with SJS/TEN, and should be done in an intensive care unit or burn unit 2.
- Supportive care is the cornerstone of management, and includes measures such as fluid resuscitation, prevention of hypothermia, nutritional support, pain and psychological distress management, and local skincare 3.
Treatment Options
- Various pharmacologic agents have been used to halt disease progression and improve outcomes, including systemic corticosteroids, intravenous immunoglobulins (IVIG), cyclosporin, and tumor necrosis factor-alpha (TNF-alpha) inhibitors 4, 5.
- However, no single drug has been found to be superior or beneficial for all patients, and the certainty of the evidence for disease-specific mortality is often very low 5.
- Etanercept, a TNF-alpha inhibitor, may reduce disease-specific mortality compared to corticosteroids, but the evidence is of low certainty 5.
- Cyclosporin may also be effective in reducing disease-specific mortality, but the evidence is very limited 5.
Consensus and Guidelines
- An international, multidisciplinary Delphi-based consensus has been established to standardize recommendations regarding supportive care in the acute phase of SJS/TEN 3.
- A guideline for the diagnosis and treatment of epidermal necrolysis (SJS/TEN) has been developed, which provides an evidence-based framework to support clinical decision-making 6.
- The guideline is aimed at specialists in various fields, as well as patients, their relatives, insurance funds, and policymakers 6.