What is the emergency treatment for Stevens-Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN) secondary to lamotrigine (Lamictal)?

Emergency Treatment for Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Secondary to Lamotrigine

Patients with SJS/TEN secondary to lamotrigine should be immediately transferred to a burn center or ICU with experience treating SJS/TEN and facilities to manage extensive skin loss wound care. 1

Immediate Actions

• Discontinue lamotrigine immediately as it is one of the most common drugs causing SJS/TEN 1, 2
• Calculate SCORTEN within the first 24 hours of admission to predict mortality risk 1
• Transfer patients with >10% body surface area (BSA) epidermal detachment to a specialized burn unit or ICU 1
• Establish peripheral venous access through non-lesional skin for fluid resuscitation 1, 2
• Arrange ophthalmology consultation within 24 hours of diagnosis to prevent permanent visual impairment 2, 3

Fluid Management

• Monitor fluid balance carefully and catheterize if clinically indicated 1
• Establish adequate intravenous fluid replacement guided by urine output and other end-point measurements 1
• Avoid overaggressive fluid resuscitation which may cause pulmonary, cutaneous, and intestinal edema 1
• Consider using the formula: body weight/% BSA epidermal detachment to determine replacement volumes 1

Wound Care

• Handle skin carefully to minimize shearing forces and further epidermal detachment 1
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the whole epidermis, including denuded areas 1
• Consider aerosolized formulations to minimize shearing forces associated with topical applications 1
• Apply nonadherent dressings (such as Mepitel™ or Telfa™) to denuded dermis 1
• Use secondary foam or burn dressings to collect exudate 1
• Take swabs for bacterial and candidal culture from lesional skin, particularly sloughy areas 1

Pain Management

• Use validated pain assessment tools at least once daily 1
• Administer adequate analgesia using intravenous opioid infusions for those not tolerating oral medication 1
• Consider patient-controlled analgesia where appropriate 1
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 1

Infection Prevention and Management

• Monitor for signs of systemic infection (confusion, hypotension, reduced urine output, reduced oxygen saturation) 1
• Do not administer prophylactic systemic antibiotics as this may increase skin colonization, particularly with Candida albicans 1
• Only institute antimicrobial therapy if there are clinical signs of infection 1
• Watch for monoculture of organisms on culture swabs from multiple sites, which indicates increased likelihood of invasive infection 1

Systemic Treatment Options

• Consider intravenous methylprednisolone for severe SJS/TEN, as it is FDA-approved for severe erythema multiforme (Stevens-Johnson syndrome) 4
• When administering high-dose methylprednisolone (30 mg/kg), infuse over at least 30 minutes and repeat every 4-6 hours for 48 hours if needed 4
• Other potential treatments include cyclosporine A and intravenous immunoglobulins, though evidence is less established 5, 6

Multidisciplinary Approach

• Coordinate care through a multidisciplinary team led by a specialist in skin failure (dermatology/plastic surgery) 1
• Include clinicians from intensive care, ophthalmology, and specialist skincare nursing 1
• Consider additional input from respiratory medicine, gastroenterology, gynaecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy as needed 1

Environmental Considerations

• Place patient in a side room controlled for humidity 1
• Use a pressure-relieving mattress 1
• Raise ambient temperature to between 25°C and 28°C 1

Common Pitfalls to Avoid

• Delayed transfer to a specialized unit increases mortality risk 1
• Overaggressive fluid resuscitation can cause complications 1
• Indiscriminate use of prophylactic antibiotics may increase skin colonization 1
• Neglecting eye care may result in permanent visual impairment 2, 3
• Failure to recognize and treat sepsis, which is the most common cause of death in SJS/TEN 1, 7

Early recognition, immediate discontinuation of lamotrigine, and prompt transfer to a specialized unit with multidisciplinary care are crucial for improving survival and reducing long-term complications in patients with SJS/TEN.