Emergency Treatment for Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

Immediate Actions

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, 2
Discontinue lamotrigine immediately as it is one of the most common drugs causing SJS/TEN 2
Calculate SCORTEN within the first 24 hours of admission to predict mortality risk 1, 2
Transfer patients with >10% body surface area (BSA) epidermal detachment to a specialized burn unit or ICU 1, 3

Monitor fluid balance carefully and catheterize if clinically indicated 4
Establish adequate intravenous fluid replacement guided by urine output and other end-point measurements 4
Avoid overaggressive fluid resuscitation which may cause pulmonary, cutaneous, and intestinal edema 3
Consider using the formula: body weight/% BSA epidermal detachment to determine replacement volumes 3

Handle skin carefully to minimize shearing forces and further epidermal detachment 5, 4
Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 3
Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the whole epidermis, including denuded areas 3
Consider aerosolized formulations to minimize shearing forces associated with topical applications 3
Apply nonadherent dressings (such as Mepitel™ or Telfa™) to denuded dermis 3
Use secondary foam or burn dressings to collect exudate 3
Take swabs for bacterial and candidal culture from lesional skin, particularly sloughy areas 4

Use validated pain assessment tools at least once daily 4
Administer adequate analgesia using intravenous opioid infusions for those not tolerating oral medication 4
Consider patient-controlled analgesia where appropriate 4
Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 4

Monitor for signs of systemic infection (confusion, hypotension, reduced urine output, reduced oxygen saturation) 5
Do not administer prophylactic systemic antibiotics as this may increase skin colonization, particularly with Candida albicans 5
Only institute antimicrobial therapy if there are clinical signs of infection 5
Watch for monoculture of organisms on culture swabs from multiple sites, which indicates increased likelihood of invasive infection 5

Coordinate care through a multidisciplinary team led by a specialist in skin failure (dermatology/plastic surgery) 2
Include clinicians from intensive care, ophthalmology, and specialist skincare nursing 2
Consider additional input from respiratory medicine, gastroenterology, gynaecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy as needed 2

Delayed transfer to a specialized unit increases mortality risk 1
Overaggressive fluid resuscitation can cause complications 3
Indiscriminate use of prophylactic antibiotics may increase skin colonization 5
Failure to recognize and treat sepsis, which is the most common cause of death in SJS/TEN 5

The British Journal of Dermatology recommends immediate discontinuation of any potential culprit drug, prompt diagnostic workup, and transfer to a specialized unit for multidisciplinary supportive care in patients with SJS/TEN 6, 7
Document symptoms suggestive of SJS/TEN, including prodromal illness, painful rash, and involvement of mucosal sites, and record the rash's progression and the index date (onset of first symptom) 8
The British Journal of Dermatology suggests documenting all medications taken over the previous 2 months, including OTC and complementary therapies, and creating a timeline for each drug 6, 9
Record vital signs, oxygen saturation, and baseline body weight, and examine for target lesions, purpuric macules, blisters, and areas of epidermal detachment 7
Calculate SCORTEN (severity-of-illness score for toxic epidermal necrolysis) to predict mortality risk 6

The British Journal of Dermatology recommends full blood count, ESR, C-reactive protein, urea and electrolytes, magnesium, phosphate, bicarbonate, glucose, liver function tests, and coagulation studies in patients with SJS/TEN 6
Take a biopsy from lesional skin adjacent to a blister for histopathology, and a second biopsy from periblister lesional skin for direct immunofluorescence to exclude immunobullous disorders 6
Histopathology typically shows epidermal necrosis, basal cell vacuolar degeneration, and subepidermal vesicle formation 10, 8

Discontinue any potential culprit drug causing SJS/TEN immediately, and establish peripheral venous access and initiate appropriate intravenous fluid resuscitation 6, 7, 11
Arrange ophthalmology consultation within 24 hours of diagnosis, and use silicone dressings for eroded areas 11, 12
Consider cyclosporine as a potential treatment option under specialist supervision 13

Daily assessment of vital signs and organ function, and regular monitoring for infection, the most common cause of mortality, are crucial in patients with SJS/TEN 6
Daily review of mucosal sites (oral, ocular, urogenital) is necessary to prevent complications 12
Neglecting eye care may result in permanent visual impairment, and overlooking infectious causes, particularly mycoplasma in children and young adults, can worsen prognosis 11

Provide written information about drug(s) to avoid, and encourage wearing a MedicAlert bracelet, and document drug allergy in patient's notes and inform all healthcare providers 13
Report the episode to pharmacovigilance authorities, and arrange follow-up with dermatology and ophthalmology within weeks of discharge 13