From the Guidelines
Management of a patient with a positive Nikolsky sign in Stevens-Johnson Syndrome (SJS) requires immediate hospitalization, preferably in a burn unit or intensive care setting. The first critical step is discontinuation of all suspected causative medications. Supportive care forms the cornerstone of management, including fluid and electrolyte replacement (typically 2-3 L/day of crystalloid fluids), temperature regulation, nutritional support (30-35 kcal/kg/day), and pain control with opioid analgesics. Wound care involves gentle cleansing with antiseptic solutions, application of non-adherent dressings, and avoiding adhesive materials. Ophthalmologic consultation is essential, with treatment including lubricating eye drops every 2 hours, topical antibiotics, and possibly topical corticosteroids. Oral care with antiseptic mouthwashes and topical anesthetics helps manage mucositis. Systemic therapy may include intravenous immunoglobulin (IVIG) at 1-2 g/kg over 3-5 days, though evidence is mixed 1. Prophylactic antibiotics are not recommended, but infections should be promptly treated when identified. The positive Nikolsky sign (skin slippage with gentle lateral pressure) indicates active disease with dermal-epidermal separation, signaling severe cutaneous involvement requiring this aggressive multidisciplinary approach to reduce mortality and prevent long-term complications.
Some key considerations in the management of SJS include:
- Handling the skin carefully to minimize epidermal detachment 1
- Limiting epidermal trauma by avoiding adhesive dressings and other irritants 1
- Using soft silicone tapes to attach essential clinical items 1
- Considering faecal management systems in immobile patients with diarrhoea 1
- Taking regular swabs for bacterial and candidal culture from areas of lesional skin 1
The most recent and highest quality study on this topic is from 2021, which provides guidance on the management of immune-related adverse events in patients treated with immune checkpoint inhibitor therapy, including SJS 1. This study recommends a multidisciplinary approach to management, including supportive care, wound care, and systemic therapy as needed.
Overall, the management of SJS requires a comprehensive and multidisciplinary approach to reduce morbidity and mortality and prevent long-term complications.
From the FDA Drug Label
Dermatologic Diseases Bullous dermatitis herpetiformis, exfoliative erythroderma, mycosis fungoides, pemphigus, severe erythema multiforme (Stevens-Johnson syndrome).
The management approach for a patient with a positive Stevens-Johnson (Stevens-Johnson Syndrome) Nikolsky sign may involve the use of corticosteroids, such as those described in the drug label 2.
- The drug label indicates that corticosteroids are used for the treatment of severe erythema multiforme (Stevens-Johnson syndrome).
- However, the label does not provide specific guidance on the management of the Nikolsky sign.
- Given the severity of Stevens-Johnson syndrome, a conservative clinical decision would be to use corticosteroids as part of the treatment approach, while closely monitoring the patient's condition.
From the Research
Management Approach for Stevens-Johnson Syndrome
The management of a patient with a positive Stevens-Johnson (Stevens-Johnson Syndrome) Nikolsky sign requires a multidisciplinary approach. Key aspects of management include:
- Immediate withdrawal of potentially causative drugs, as they are considered a medical emergency and early recognition and appropriate management is decisive for survival 3, 4
- Prompt referral to an appropriate medical center for specific supportive treatment, which is of utmost importance 3
- Supportive care, which is the most universally accepted intervention for SJS/TEN, and includes measures such as wound care and management of mucous membrane lesions 5
- Consideration of immunomodulating agents, such as systemic corticosteroids, immunoglobulins, and cyclosporine A, although the efficacy of these treatments remains uncertain 3, 6, 5
Treatment Options
Treatment options for SJS/TEN include:
- Systemic corticosteroids, which remain one of the most common treatment options, although their efficacy is uncertain 6, 5
- Cyclosporine, which has shown promise in decreasing mortality, but requires further study to confirm efficacy and safety 6
- TNF-α inhibitors, which have also shown promise in decreasing mortality, but require further study to confirm efficacy and safety 6
- Intravenous immunoglobulin (IVIG), which is currently the standard of care for most referral centers, but has conflicting data regarding its effectiveness 5
Important Considerations
Important considerations in the management of SJS/TEN include:
- Early recognition and prompt management, as this can be lifesaving 3, 4, 7
- Identification and withdrawal of the culprit drug, which is crucial in preventing further progression of the disease 3, 4
- Multidisciplinary care, which includes supportive care, medical treatment, communication, and provision of appropriate information and emotional support 7