What is the treatment for Stephen Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

The most effective treatment for Stevens-Johnson syndrome requires 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 and guide treatment decisions 1, 3
• Transfer to a specialized care center, preferably a burn unit or ICU for patients with >10% body surface area epidermal detachment 1, 2
• Place patient in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 2
• Establish careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload 1, 2

Wound Care

• Minimize shearing forces when handling skin to prevent further epidermal detachment 4
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 4
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the whole epidermis, including denuded areas 4
• Leave detached epidermis in situ to act as a biological dressing 4
• Apply nonadherent dressings (such as Mepitel™ or Telfa™) to denuded dermis 4
• Use secondary foam or burn dressings to collect exudate 4

Mucosal Care

Ocular Care

• Arrange urgent ophthalmology review within 24 hours of diagnosis 4
• Perform daily ophthalmology review during the acute phase 4
• Maintain daily ocular hygiene with gentle saline irrigation to remove mucous or debris 4
• Apply preservative-free lubricant eye drops every two hours throughout the acute illness 1
• Remove pseudomembranes and break down conjunctival adhesions 4
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1, 2

Oral Care

• Perform daily oral review during the acute illness 4
• Apply white soft paraffin ointment to the lips every 2 hours 4
• Clean the mouth daily with warm saline mouthwashes or an oral sponge 4
• Use an anti-inflammatory oral rinse containing benzydamine hydrochloride every 2-4 hours, particularly before eating 4
• Consider a potent topical corticosteroid mouthwash (e.g., betamethasone sodium phosphate) four times daily 4

Urogenital Care

• Perform daily urogenital review during the acute illness 4
• Apply white soft paraffin ointment to the urogenital skin and mucosae every 4 hours 4
• Consider catheterization if required to reduce pain on passing urine and for assessment of fluid balance 4
• Use a potent topical corticosteroid ointment once daily to involved, noneroded surfaces 4
• Use silicone dressings (e.g., Mepitel) to eroded areas 4

Systemic Therapy Options

• Systemic corticosteroids, particularly early methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 1, 2, 5
• Cyclosporine (3-5 mg/kg/day) for 10-14 days has shown benefit with reduced mortality compared to predicted rates 1, 6
• For severe cases, consider intravenous immunoglobulin as an alternative therapy 6, 7

Infection Prevention and Management

• Monitor for signs of infection rather than using prophylactic antibiotics 2, 3
• Take regular skin swabs for culture to detect predominant organisms 2
• Only institute antimicrobial therapy when clinical signs of infection are present 2, 3

Airway Management

• Respiratory symptoms and hypoxemia on admission should prompt early discussion with an intensivist 4
• Consider fibreoptic bronchoscopy to assess airway involvement 4

Discharge and Follow-up

• Provide written information about drug(s) to avoid 4, 1
• Encourage the patient to wear a MedicAlert bracelet 4, 1
• Document drug allergy in the patient's notes and inform all doctors involved in the patient's care 4, 1
• Report the episode to national pharmacovigilance authorities 4, 1
• Organize a dermatology outpatient clinic appointment, and if required, an ophthalmology outpatient appointment, within a few weeks of discharge 4, 1

Common Pitfalls to Avoid

• Delayed recognition and discontinuation of the culprit medication significantly increases mortality risk 1, 8
• Indiscriminate use of prophylactic antibiotics may increase skin colonization with resistant organisms 2, 3
• Overaggressive fluid resuscitation can lead to pulmonary, cutaneous, and intestinal edema 4, 2
• Failure to involve appropriate specialists early, particularly ophthalmology, can lead to permanent sequelae 1, 3