Treatment of Stevens-Johnson Syndrome (SJS)

Initial Management

The British Journal of Dermatology recommends 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
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 3, 2
Careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload 2

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

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

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, 4
Use an antiseptic oral rinse containing chlorhexidine twice daily 1, 4

Daily urogenital review during the acute illness 1
Apply white soft paraffin ointment to the urogenital skin and mucosae every 4 hours 1, 4
Use a potent topical corticosteroid ointment once daily to involved, noneroded surfaces 1, 4
Use silicone dressings (e.g., Mepitel) to eroded areas 1, 4

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

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

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

Delayed recognition and discontinuation of the culprit medication significantly increases mortality risk 2
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