What is the treatment for Steven Johnson syndrome?

Treatment of Stevens-Johnson Syndrome (SJS)

The most critical first step in treating Stevens-Johnson Syndrome is immediate discontinuation of any potential culprit drug, followed by transfer to a specialized care center with experience managing SJS/TEN, preferably a burn unit or ICU for patients with >10% body surface area epidermal detachment. 1, 2

Initial Management

• Assess severity using SCORTEN to predict mortality risk 2
• Transfer to a specialized care center with experience managing SJS/TEN, preferably a burn unit or ICU for patients with >10% body surface area epidermal detachment 1, 2
• Barrier nursing in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 2
• Careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload 2

Supportive Care

Wound Management

• Minimize shearing forces when handling skin to prevent further epidermal detachment 2
• Apply bland emollients frequently to support barrier function and reduce water loss 2
• Leave detached epidermis in situ to act as a biological dressing 2
• Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings 2
• Consider silver-containing products/dressings for sloughy areas only 2, 3

Infection Prevention

• Monitor for signs of infection rather than using prophylactic antibiotics 2
• Regular skin swabs for culture to detect predominant organisms 2
• Targeted antimicrobial therapy only when clinical signs of infection are present 2

Mucosal Management

Ocular Care

• Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews during acute illness 1, 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
• Administer broad-spectrum topical antibiotics when corneal fluorescein staining or ulceration is present 1, 2
• Consider topical corticosteroids for ocular inflammation under ophthalmologist supervision 2

Oral Care

• Daily oral review during the acute illness 1
• Apply white soft paraffin ointment to the lips every 2 hours 1
• Clean the mouth daily with warm saline mouthwashes or an oral sponge 1
• Use an anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1
• Use an antiseptic oral rinse containing chlorhexidine twice daily 1
• Use a potent topical corticosteroid mouthwash (e.g., betamethasone sodium phosphate) four times daily 1
• Consider topical anesthetics such as viscous lidocaine 2% for severe oral discomfort 1

Urogenital Care

• Daily urogenital review during the acute illness 1
• Apply white soft paraffin ointment to the urogenital skin and mucosae every 4 hours 1
• Use a potent topical corticosteroid ointment once daily to involved, noneroded surfaces 1
• Use silicone dressings (e.g., Mepitel) to eroded areas 1
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1
• Catheterize patients to prevent urethral strictures 1

Systemic Therapy Options

• Ciclosporin (3 mg/kg daily for 10 days, tapered over 1 month) has shown benefit 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
• Intravenous immunoglobulin (IVIG) has been used with some success in certain centers 5

Airway Management

• Respiratory symptoms and hypoxemia on admission should prompt early discussion with an intensivist and rapid transfer to an ICU or burn center 1
• Fibreoptic bronchoscopy should be undertaken to assess airway involvement 1

Discharge and Follow-up

• Provide written information about drug(s) to avoid 1, 2
• Encourage the patient to wear a MedicAlert bracelet 1, 2
• Document drug allergy in the patient's notes; inform all doctors involved in the patient's care 1, 2
• Report the episode to national pharmacovigilance authorities 1, 2
• Organize a dermatology outpatient clinic appointment, and if required, an ophthalmology outpatient appointment, within a few weeks of discharge 1

Common Pitfalls to Avoid

• Delayed recognition and discontinuation of the culprit medication significantly increases mortality risk 2, 4
• Indiscriminate use of prophylactic antibiotics may increase skin colonization with resistant organisms 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