Immediate Treatment for Stevens-Johnson Syndrome
Immediately discontinue all potential culprit medications and transfer the patient to a specialized burn unit or ICU with experience managing SJS/TEN, while initiating aggressive supportive care focused on fluid resuscitation, wound management, and prevention of complications. 1, 2
Critical First Actions
Stop the offending drug immediately – this is the single most important intervention that directly impacts mortality. 1, 3 Document all medications taken in the preceding 2 months, including over-the-counter and herbal products, and use the ALDEN algorithm to identify the likely culprit. 1
Calculate SCORTEN within 24 hours to predict mortality risk and guide intensity of care. 1, 2, 3 This severity score helps determine appropriate level of monitoring and intervention.
Transfer to specialized care – patients with >10% body surface area (BSA) epidermal detachment require immediate transfer to a burn center or ICU with multidisciplinary expertise in SJS/TEN management. 1, 2 Delayed transfer significantly increases mortality risk. 2
Immediate Supportive Care
Fluid Management
Establish intravenous access and initiate careful fluid resuscitation guided by urine output (target 0.5-1 mL/kg/hour) and vital signs. 1, 2, 3 Use the formula: body weight/% BSA epidermal detachment to determine replacement volumes. 2
Avoid overaggressive fluid resuscitation – this is a critical pitfall that causes pulmonary, cutaneous, and intestinal edema. 2, 3 Monitor fluid balance meticulously with urinary catheterization if clinically indicated. 2
Wound Care
Handle skin with extreme gentleness to minimize shearing forces and prevent further epidermal detachment. 2, 3 Leave detached epidermis in place to act as a biological dressing. 3
Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis, including denuded areas. 2, 3 Consider aerosolized formulations to minimize trauma. 2
Use nonadherent dressings (Mepitel™ or Telfa™) on denuded dermis with secondary foam or burn dressings to collect exudate. 2, 3 Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000). 2, 3
Environmental Control
Place patient in a temperature-controlled room maintained at 25-28°C on a pressure-relieving mattress. 2, 3 This reduces insensible water losses and prevents pressure injuries.
Pain Management
Administer adequate analgesia using intravenous opioid infusions for patients unable to tolerate oral medication. 2 Use validated pain assessment tools at least once daily. 2 Consider patient-controlled analgesia or sedation/general anesthesia for dressing changes and patient repositioning. 2
Infection Prevention
Do NOT give prophylactic systemic antibiotics – this is a common error that increases skin colonization, particularly with Candida albicans. 2, 3 Instead, monitor closely for signs of systemic infection (confusion, hypotension, reduced urine output, decreased oxygen saturation). 2
Take bacterial and fungal swabs from lesional skin, particularly sloughy areas, for culture and sensitivity. 1, 2 Institute targeted antimicrobial therapy only when clinical signs of infection develop. 2, 3 Watch for monoculture of organisms on swabs from multiple sites, indicating increased likelihood of invasive infection. 2
Critical Specialist Consultations
Arrange ophthalmology examination within 24 hours by a specialist experienced in ocular surface diseases. 1, 2, 3 This is non-negotiable – failure to provide early eye care results in permanent visual impairment. 3 Daily ophthalmologic reviews are required throughout the acute illness. 3
Establish multidisciplinary team led by a dermatologist or plastic surgeon with expertise in skin failure, including intensive care, ophthalmology, and specialist skincare nursing. 2, 3 Additional specialists (respiratory medicine, gastroenterology, gynecology, urology, oral medicine) should be consulted based on organ involvement. 2
Initial Investigations
Order comprehensive laboratory workup: full blood count, C-reactive protein, urea and electrolytes, liver function tests, coagulation studies, glucose, magnesium, phosphate, bicarbonate, base excess, and lactate. 1, 2
Obtain skin biopsy from lesional skin adjacent to a blister for histopathology (showing epidermal necrosis and subepidermal vesicle formation), and a second periblister biopsy for direct immunofluorescence to exclude immunobullous disorders. 3
Screen for infectious triggers including mycoplasma and chlamydia serology, skin swabs for HSV and varicella zoster virus, chest X-ray, and conjunctival swabs for bacteria, chlamydia, HSV, and adenovirus. 1, 2 This is particularly important in children and young adults where mycoplasma may be causative. 1
Systemic Immunomodulation Considerations
While supportive care is the cornerstone of management, systemic corticosteroids (IV methylprednisolone 1-2 mg/kg) may be beneficial if initiated within 72 hours of onset. 1, 3, 4 The British Association of Dermatologists guidelines note that early methylprednisolone pulse therapy shows potential benefit. 3
Cyclosporine (3 mg/kg daily for 10 days) has shown reduced mortality in multiple studies and is recommended by the British Journal of Dermatology. 3, 4 However, these interventions should ideally be administered under specialist supervision in the context of a clinical study or registry. 1
Common Pitfalls to Avoid
- Delayed recognition and transfer to specialized units dramatically increases mortality 2
- Continuing the culprit medication even briefly worsens outcomes 3
- Indiscriminate prophylactic antibiotics promote resistant organism colonization 2, 3
- Overaggressive fluid resuscitation causes life-threatening edema 2, 3
- Neglecting early ophthalmology consultation results in permanent visual impairment 3
- Failure to recognize sepsis, which is the most common cause of death in SJS/TEN 2