What is the management of Stevens-Johnson syndrome?

Management of Stevens-Johnson Syndrome

Patients with Stevens-Johnson syndrome (SJS) with >10% body surface area epidermal loss should be immediately transferred to a burn center or intensive care unit with experience in treating SJS/TEN and facilities to manage extensive skin loss wound care. 1

Initial Assessment and Management

• Immediately identify and discontinue any potential culprit drug(s) as the first and most critical step 2
• Calculate SCORTEN within the first 24 hours to predict mortality risk and guide management intensity 2, 3
• Admit patients to a specialized care setting with barrier nursing in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 1
• Assemble a multidisciplinary team led by a specialist in skin failure, including intensive care, ophthalmology, and specialist skincare nursing 2

Skin and Wound Management

• Minimize shearing forces when handling skin to prevent further epidermal detachment 1
• Apply bland emollients frequently to support barrier function and reduce transcutaneous water loss 1, 2
• Leave detached epidermis in situ to act as a biological dressing 2, 3
• Gently cleanse wounds by irrigating with warmed sterile water, saline, or dilute chlorhexidine 2, 3
• Apply non-adherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate 2
• Consider silver-containing products for sloughy areas only 2

Infection Prevention and Management

• Monitor for signs of infection rather than using prophylactic antibiotics, which may increase skin colonization with resistant organisms 1, 2
• Obtain regular skin swabs for culture to detect predominant organisms 2, 3
• Institute targeted antimicrobial therapy only when clinical signs of infection are present 1, 2
• Be vigilant for signs of systemic infection (confusion, hypotension, reduced urine output) as sepsis is the most common cause of death 1, 3

Mucosal Management

Ocular Care

• Arrange ophthalmological examination within 24 hours of diagnosis and continue daily reviews during the acute illness 2
• Apply preservative-free lubricant eye drops every two hours throughout the acute illness 2
• Perform daily ocular hygiene to remove inflammatory debris and break down conjunctival adhesions 2
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 2
• Consider topical corticosteroids for ocular inflammation under ophthalmologist supervision 2

Oral Care

• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 2
• Apply antiseptic oral rinse twice daily 2
• Use topical anesthetics such as viscous lidocaine 2% for severe oral discomfort 2
• Apply topical corticosteroids to oral mucosa if needed 2

Urogenital Care

• Consider urinary catheterization when urogenital involvement causes dysuria or retention 2, 3
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 2
• Consider vaginal dilators or tampons wrapped in non-adherent dressings to prevent vaginal synechiae formation 2

Supportive Care

• Carefully monitor fluid management with regular assessment of vital signs, urine output, and electrolytes 2, 3
• Provide continuous enteral nutrition throughout the acute phase, delivering 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery 2
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 2
• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 2

Systemic Therapy Options

• Consider cyclosporine (3 mg/kg daily for 10 days, tapered over 1 month) as it has shown benefit in multiple studies with reduced mortality compared to predicted rates 2
• Systemic corticosteroids, particularly early methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 2, 4

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 2, 3
• Encourage patients to wear a MedicAlert bracelet or amulet bearing the name of the culprit drug 2
• Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care 2, 3
• Report the adverse drug reaction to pharmacovigilance authorities 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 2

Common Pitfalls to Avoid

• Delayed recognition and treatment significantly increases mortality risk 2, 5
• Indiscriminate use of prophylactic antibiotics may increase skin colonization with resistant organisms 1, 2
• Overaggressive fluid resuscitation can lead to pulmonary, cutaneous, and intestinal edema 2
• Failure to involve appropriate specialists early, particularly ophthalmology, can lead to permanent sequelae 2, 6
• Continued use of the culprit medication will worsen the condition and increase mortality 2, 7