What is the treatment for Stevens-Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

The management of Stevens-Johnson Syndrome (SJS) requires immediate withdrawal of the suspected causative drug, transfer to a specialized center for severe cases, and comprehensive supportive care focusing on wound management, infection prevention, and mucosal care. 1

Initial Assessment and Management

• Patient Transfer: Patients with confirmed Toxic Epidermal Necrolysis (TEN) (>30% skin detachment), SJS/TEN overlap with poor prognostic factors, or severe eye disease should be transferred to a specialized center 1
• Risk Assessment: Calculate SCORTEN within the first 24 hours to assess mortality risk 1
• Drug Withdrawal: Immediately discontinue any suspected causative medication 1, 2

Supportive Care

Wound Management

• Gently cleanse wounds using warmed sterile water, saline, or dilute chlorhexidine (1/5000) 1
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis 1
• Leave detached epidermis in situ as a biological dressing 1
• Apply non-adherent dressings to denuded dermis (Mepitel™ or Telfa™) 1
• Handle skin carefully to minimize further epidermal detachment 1

Fluid Management

• Establish adequate intravenous fluid replacement through non-lesional skin 1
• Monitor fluid balance carefully, catheterizing if clinically indicated 1
• Use continuous invasive hemodynamic monitoring in severe cases 1
• Be cautious of overhydration and resultant hyponatremia 1

Infection Prevention

• Take swabs for bacterial and candidal culture from lesional skin throughout the acute phase 1
• Apply topical antimicrobial agents only to sloughy areas 1
• Do not administer prophylactic systemic antibiotics; only treat when clinical signs of infection are present 1
• Be vigilant for sepsis, which may be masked by disease-associated fever 1

Mucosal Care

Ocular Management

• Perform daily ophthalmological review during acute illness 1
• Apply preservative-free lubricants every 2 hours 1
• Use topical antibiotics if corneal fluorescein staining or ulceration is present 3, 1
• Consider topical corticosteroid drops (nonpreserved dexamethasone 0.1%) under ophthalmologist supervision 3, 1
• Prevent corneal exposure in unconscious patients using a moisture chamber with polyethylene film 3, 1

Oral Care

• Clean the mouth daily with warm saline mouthwashes 1
• Use benzydamine hydrochloride rinse every 3 hours, particularly before eating 1
• Consider topical anesthetic preparations (viscous lidocaine 2%) for severe pain 1
• Use antiseptic oral rinse twice daily (hydrogen peroxide 1.5% or chlorhexidine 0.2%) 1

Urogenital Care

• Examine the urogenital tract as part of the initial assessment 1
• Apply white soft paraffin to urogenital skin/mucosae every 4 hours 1
• Use silicone dressings on eroded areas to reduce pain and prevent adhesions 1
• Consider potent topical corticosteroid ointment on non-eroded surfaces 1

Pharmacological Management

Systemic Treatments

• For immune checkpoint inhibitor-induced SJS/TEN, administer IV methylprednisolone 0.5-1 mg/kg for Grade 3, and 1-2 mg/kg for Grade 4 1
• Consider moderate to high doses of oral or parenteral corticosteroids (prednisolone 1-2 mg/kg/day or equivalent), preferably within 72 hours of onset, tapered rapidly within 7-10 days 2
• Cyclosporine (3-5 mg/kg/day) for 10-14 days may be used either alone or in combination with corticosteroids 2
• Intravenous immunoglobulin (IVIG) may be considered in severe or steroid-unresponsive cases 4, 5

Pain Management

• Use appropriate validated pain assessment tools at least once daily 1
• Administer adequate analgesia using intravenous opioid infusions if oral medication is not tolerated 1
• Consider patient-controlled analgesia where appropriate 1
• Consider sedation or general analgesia for painful procedures 1

Follow-up Care

• Provide written information about drugs to avoid 1
• Encourage wearing a MedicAlert bracelet 1
• Document drug allergy clearly in patient records 1
• Report the episode to pharmacovigilance authorities 1
• Arrange dermatology and ophthalmology follow-up within weeks of discharge 1

Cautions and Pitfalls

• Avoid indiscriminate use of prophylactic antibiotics, as this may increase skin colonization 1
• Avoid adhesive dressings, ECG leads, and identification wrist tags that can cause further skin trauma 1
• Be aware that systemic corticosteroids may increase the risk of infection 3
• Recognize that while corticosteroids may suppress disease progression, they also enhance infection risk, which is the most common cause of mortality 6

The management of SJS requires a multidisciplinary approach with careful attention to supportive care, which has been shown to reduce mortality rates to as low as 10% in specialized centers 5.