What is the immediate treatment for a patient diagnosed with Stevens-Johnson syndrome?

Immediate Treatment for Stevens-Johnson Syndrome

The immediate treatment for Stevens-Johnson syndrome requires prompt discontinuation of any potential culprit drug, transfer to a specialized burn unit or ICU, and implementation of comprehensive supportive care with a multidisciplinary approach. 1, 2

Initial Management

• Immediately discontinue any potential culprit drug causing SJS/TEN 2, 1
• Transfer patients with >10% body surface area epidermal detachment to a specialized burn unit or ICU with experience treating SJS/TEN 1, 2
• Calculate SCORTEN within the first 24 hours of admission to predict mortality risk 1, 2
• Place patient in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 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

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 1
• Apply nonadherent dressings (such as Mepitel™ or Telfa™) to denuded dermis 1
• Use secondary foam or burn dressings to collect exudate 1

Infection Management

• Monitor for signs of systemic infection (confusion, hypotension, reduced urine output, reduced oxygen saturation) 1
• Take swabs for bacterial and candidal culture from lesional skin, particularly sloughy areas 1
• Do not administer prophylactic systemic antibiotics as this may increase skin colonization 1
• Only institute antimicrobial therapy if there are clinical signs of infection 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

Mucosal Management

Ocular Care

• Arrange ophthalmology consultation within 24 hours of diagnosis 2, 1
• Apply frequent lubrication with preservative-free artificial tears 3
• Daily examination for symblepharon formation 3
• Lysis of adhesions performed by an ophthalmologist to prevent permanent scarring 3

Oral Care

• Anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours 3
• Antiseptic oral rinse twice daily 3
• Topical anesthetics for severe oral discomfort 3

Urogenital Care

• Consider urinary catheterization when urogenital involvement causes dysuria or retention 3
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 3
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 3

Systemic Therapy Options

• Systemic corticosteroids may be beneficial if started early, preferably within 72 hours 3, 2
• Consider IV methylprednisolone (or equivalent) 0.5-1 mg/kg and convert to oral corticosteroids on response, weaning over at least 4 weeks 2
• Cyclosporine may be considered as a treatment option under specialist supervision 1, 4

Laboratory Investigations

• Order full blood count, C-reactive protein, urea and electrolytes, liver function tests, coagulation studies, glucose, magnesium, phosphate, bicarbonate, base excess, and lactate 2
• Perform infection screening including mycoplasma and chlamydia serology, skin swabs for HSV and varicella zoster virus 2
• Take bacterial swabs from lesional skin for culture and sensitivity 2
• Collect conjunctival swabs for bacteria, chlamydia, HSV, and adenovirus 2

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, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy as needed 1

Common Pitfalls to Avoid

• Delayed recognition and transfer to a specialized unit significantly increases mortality risk 1, 5
• Indiscriminate use of prophylactic antibiotics may increase skin colonization with resistant organisms 1
• Failure to involve ophthalmology early can lead to permanent visual impairment 1
• Continued use of the culprit medication will worsen the condition and increase mortality 1, 6
• Overaggressive fluid resuscitation can lead to pulmonary, cutaneous, and intestinal edema 1