What is the treatment for Stevens-Johnson Syndrome (SJS)?

Treatment of Stevens-Johnson Syndrome (SJS)

The treatment of Stevens-Johnson Syndrome requires immediate transfer to a specialized center (burn unit or ICU) for multidisciplinary supportive care, with prompt withdrawal of any causative drugs and careful wound management to prevent complications and reduce mortality. 1

Initial Management and Triage

• Patients with >10% body surface area (BSA) epidermal loss should be transferred to a burn center or ICU 1
• Calculate SCORTEN within first 24 hours to assess mortality risk 1
• Establish adequate intravenous fluid replacement through non-lesional skin 1
• Monitor fluid balance carefully, with catheterization if clinically indicated 1
• Use continuous invasive hemodynamic monitoring in severe cases 1
• Maintain ambient temperature between 25°C and 28°C 1

Wound Management

• Gently cleanse wounds using warmed sterile water, saline, or dilute chlorhexidine (1/5000) 2, 1
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis 2, 1
• Leave detached epidermis in situ as a biological dressing 1
• Decompress blisters by piercing and draining fluid 1
• Apply non-adherent dressings to denuded dermis (Mepitel™ or Telfa™) 1
• Use secondary foam or burn dressing to collect exudate 1
• Handle skin carefully to minimize further epidermal detachment 1

Infection Prevention and Management

• Take swabs for bacterial and candidal culture from lesional skin throughout the acute phase 1
• Do not administer prophylactic systemic antibiotics - only treat when clinical signs of infection are present 2, 1
• Apply topical antimicrobial agents only to sloughy areas 1
• Consider silver-containing products/dressings (limited use if extensive areas) 1
• Be vigilant for sepsis, which may be masked by disease-associated fever 2, 1
• Monitor for respiratory symptoms and hypoxemia 1

Ocular Management

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

Mucosal 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
• 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 (Mepitel) on eroded areas to reduce pain and prevent adhesions 1

Pain Management

• Administer adequate background simple analgesia following WHO analgesic ladder principles 2
• For moderate-to-severe pain uncontrolled by simple analgesia, use opiate-based regimen with morphine 2
• Consider patient-controlled analgesia (PCA) if appropriate 2, 1
• Use intravenous opioid infusions if oral medication is not tolerated 1
• Monitor level of consciousness, respiratory rate and oxygen saturation when using opiate infusions 2
• Consider sedation or general analgesia for painful procedures 1

Additional Supportive Care

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients 2
• Consider proton pump inhibitor for patients in whom enteral nutrition cannot be established 2
• Consider recombinant human G-CSF for neutropenic patients 2
• Avoid adhesive dressings, ECG leads, and identification wrist tags that can cause further skin trauma 1
• Use pressure-relieving mattress 1
• Perform regular laboratory monitoring: full blood count, electrolytes, liver function tests, glucose, magnesium, phosphate 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
• Monitor for potential complications including ocular, cutaneous, or renal sequelae 3

Cautions and Pitfalls

• Avoid indiscriminate use of prophylactic antibiotics, as this may increase skin colonization 2, 1
• Be cautious of overhydration and resultant hyponatremia 1
• Recognize that septicemia is a leading cause of morbidity and fatality in the acute phase 2
• Avoid drugs with high risk of causing SJS/TEN: anti-infective sulfonamides, anti-epileptic drugs, NSAIDs of the oxicam type, allopurinol, nevirapine 3, 4
• Remember that the most common complications are ocular, cutaneous, or renal 3