From the Guidelines
Stevens-Johnson Syndrome (SJS) is a severe skin reaction that requires immediate medical attention, and the most effective treatment involves supportive care in a hospital setting, including stopping the causative medication, fluid management, wound care, pain control, and sometimes corticosteroids or immunoglobulin therapy. The condition is characterized by widespread epithelial keratinocyte apoptosis and necrosis, a process initiated by drug-induced cytotoxic T lymphocytes (CTLs) 1. The mortality rate for SJS is less than 10%, while for Toxic Epidermal Necrolysis (TEN), it is around 30%, with an overall mortality rate of about 22% for SJS/TEN 1.
Key Aspects of Management
- Supportive care is the most important aspect of treatment, including care of skin, mucous membranes, resuscitation, fluid balance, nutritional support, analgesia, and preventing life-threatening complications and long-term morbidity 1.
- Patients with suspected SJS/TEN should be referred immediately to a multidisciplinary team (MDT) and assessed for severity using the SCORTEN score 1.
- The use of an appropriate dressing on exposed dermis can reduce fluid and protein loss, limit microbial colonization, and help with pain control 1.
- Antimicrobial therapy should only be instituted if there are clinical signs of infection, as indiscriminate administration of prophylactic systemic antibiotics may increase skin colonization 1.
Care Setting
- Children and young people with SJS/TEN should be managed in age-appropriate specialist units with an MDT, and those with limited SJS who are well may be suitably managed on an age-appropriate ward 1.
- Patients with more extensive skin loss, systemic involvement, or comorbidities should be managed in a unit that includes pediatric intensivists and specialists in extensive skin loss (burns surgeons and dermatologists) 1.
Importance of Early Transfer to Specialized Care
- Delay in transfer to specialized care can adversely affect outcomes, and high-risk children need quicker transfer to specialized care 1.
From the Research
Definition and Causes of SJS
- Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe, life-threatening mucocutaneous adverse drug reactions with a high morbidity and mortality that require immediate medical care 2.
- The exact pathomechanism of SJS/TEN remains unclear, but keratinocyte death is thought to be triggered by immune reactions to antigens, with medications being the most common cause 3, 4.
- Risk factors for the development of SJS/TEN include immune dysregulation, active malignancy, and genetic predisposition 4.
Treatment and Management of SJS
- The ideal therapy of Stevens-Johnson syndrome/toxic epidermal necrolysis still remains a matter of debate as there are only a limited number of studies of good quality comparing the usefulness of different specific treatments 2.
- Available data support the use of various pharmacologic agents to halt disease progression and improve outcomes, including systemic corticosteroids, cyclosporine, intravenous immunoglobulin, and tumor necrosis factor-α inhibitors 2, 3, 5.
- A multidisciplinary approach in the management of these patients is helpful, with all patients being managed initially in an intensive care unit or burn unit under a team of physicians experienced in the care of patients with SJS/TEN 4, 6.
- Prompt withdrawal of the culprit drug, meticulous supportive care, and judicious and early initiation of moderate to high doses of oral or parenteral corticosteroids are recommended 2.
- Cyclosporine and intravenous immunoglobulin may also be used either alone or in combination with corticosteroids to improve the prognosis of SJS/TEN 2, 3.
Prognosis and Outcomes
- The mortality rates for SJS/TEN can range from 15% to 25%, with early treatment with high-dose steroids and intravenous immunoglobulin being possible therapeutic options to improve the prognosis 3, 4.
- Complete recovery is possible with appropriate treatment, but residual skin, mucosal, or visceral damage may occur in some cases 5.