What is the management of Steven Johnson syndrome caused by a drug?

Management of Stevens-Johnson Syndrome Caused by a Drug

The most critical step in managing drug-induced Stevens-Johnson syndrome (SJS/TEN) is immediate discontinuation of the suspected culprit medication, followed by prompt transfer to a specialized unit for multidisciplinary supportive care. 1, 2

Immediate Actions

• Identify and immediately withdraw any suspected medication as this decreases the risk of death 1
• Calculate SCORTEN within the first 24 hours of admission to predict mortality risk 2
• Transfer patients with >10% body surface area epidermal detachment to a specialized burn unit or ICU with experience treating SJS/TEN 2
• Patients should be managed in age-appropriate specialist units with an appropriate multidisciplinary team 1

Diagnostic Workup

• Take a biopsy from lesional skin adjacent to a blister for histopathology to confirm diagnosis 2
• Document all medications taken over the previous 2 months, including over-the-counter and complementary therapies 2
• Use the ALDEN algorithm to help define drug causality in SJS/TEN 1
• Perform relevant testing for potential infective triggers, especially in children where infections cause up to 50% of cases 1

Supportive Care

• Establish adequate intravenous fluid replacement guided by urine output and other end-point measurements 2
• Handle skin carefully to minimize shearing forces and further epidermal detachment 2
• Apply greasy emollient over the whole epidermis, including denuded areas 2
• Use nonadherent dressings (such as Mepitel™ or Telfa™) to denuded dermis 2

Wound Care

• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine 2
• Take swabs for bacterial and candidal culture from lesional skin, particularly sloughy areas 2
• Monitor for signs of systemic infection (confusion, hypotension, reduced urine output, reduced oxygen saturation) 2
• Do not administer prophylactic systemic antibiotics as this may increase skin colonization, particularly with Candida albicans 2

Pain Management

• Use validated pain assessment tools at least once daily 2
• Administer adequate analgesia using intravenous opioid infusions for those not tolerating oral medication 2
• Consider patient-controlled analgesia where appropriate 2
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 2

Mucosal Care

• Arrange ophthalmology consultation within 24 hours of diagnosis to prevent permanent visual impairment 2
• Perform daily assessment of oral, ocular, and urogenital mucous membranes 2
• Provide appropriate care for mucosal sites to prevent long-term complications such as strictures 3

Systemic Therapy Options

• Consider cyclosporine (3 mg/kg daily for 10 days, tapered over 1 month) as a potential treatment option under specialist supervision 1
• Systemic corticosteroids remain controversial - if used, they should only be administered in the phase of progression and withdrawn as soon as possible to reduce infection risk 3
• Intravenous immunoglobulins have been used but require confirmation in larger clinical trials 3

Environmental Considerations

• Place patient in a side room controlled for humidity 2
• Use a pressure-relieving mattress 2
• Raise ambient temperature to between 25°C and 28°C 2

Follow-up and Discharge Planning

• Encourage the patient to wear a MedicAlert bracelet or amulet bearing the name of the culprit drug 1
• Document drug allergy in the patient's notes and inform all healthcare providers involved in the patient's care 1
• Report the episode to national pharmacovigilance authorities 1
• Arrange follow-up appointments with dermatology and ophthalmology within weeks of discharge 1
• Consider referral for diagnostic testing to a specialist center with expertise in drug allergy in severe cases 1

Common Pitfalls to Avoid

• Delayed transfer to a specialized unit increases mortality risk 2
• Overaggressive fluid resuscitation can cause pulmonary, cutaneous, and intestinal edema 2
• Failure to recognize and treat sepsis, which is the most common cause of death in SJS/TEN 2
• Routine drug hypersensitivity testing is not recommended following an episode of SJS/TEN 1
• Oral provocation studies are not ethical in SJS/TEN because of the risk of life-threatening reactions 1

High-Risk Medications

• Anti-infective sulfonamides, anticonvulsants (particularly carbamazepine and phenytoin), allopurinol, NSAIDs of the oxicam type, and nevirapine are most commonly implicated 4, 5
• Phenytoin specifically carries warnings about serious skin reactions including SJS/TEN, with increased risk in patients of Asian ancestry with HLA-B*1502 6
• In children, anticonvulsants and antibiotics are the most commonly implicated medications 1