What is the treatment for Steven Johnson syndrome?

Treatment of Stevens-Johnson Syndrome (SJS)

The most effective treatment for Stevens-Johnson Syndrome requires immediate discontinuation of any potential culprit drug, prompt transfer to a specialized care center, and comprehensive multidisciplinary supportive care with consideration of early systemic immunomodulatory therapy. 1, 2

Initial Management

• Immediately discontinue any suspected causative medication as this is the most critical first step in SJS management 1, 2
• Calculate SCORTEN within the first 24 hours to predict mortality risk and guide management decisions 2
• Transfer patients with >10% body surface area epidermal detachment to a specialized burn unit or ICU with experience managing SJS/TEN 1, 2
• Place patient in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 1

Supportive Care

Fluid and Nutritional Management

• Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload 1
• Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 1
• Deliver continuous enteral nutrition throughout the acute phase, providing 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery 3
• Consider nasogastric feeding when oral intake is precluded by buccal mucositis 3

Wound Care

• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 3
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis, including denuded areas 3
• Leave detached epidermis in situ to act as a biological dressing; decompress blisters by piercing and expression or aspiration of fluid 3
• Apply nonadherent dressings (e.g., Mepitel™ or Telfa™) to denuded dermis with secondary foam or burn dressings to collect exudate 3, 2
• Consider silver-containing products/dressings for sloughy areas only (limit use if extensive areas are being treated due to absorption risk) 3

Mucosal Management

Ocular Care

• Arrange ophthalmological examination within 24 hours of diagnosis and continue daily reviews during the acute illness 3, 2
• Apply preservative-free lubricant eye drops (e.g., hyaluronate or carmellose) every two hours throughout the acute illness 3
• Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 3
• Use topical antibiotics (e.g., moxifloxacin or levofloxacin) when corneal fluorescein staining or ulceration is present 3
• Consider topical corticosteroids for ocular inflammation under ophthalmologist supervision 3, 1

Oral and Genital Care

• Apply anti-inflammatory oral rinses containing benzydamine hydrochloride every 3 hours, particularly before eating 1
• Use topical anesthetics such as viscous lidocaine 2% for severe oral discomfort 1
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 1
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Pain Management

• Use validated pain assessment tools at least once daily 2
• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 3
• Consider patient-controlled analgesia where appropriate 2
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 2

Systemic Therapy Options

Immunomodulatory Therapy

• Ciclosporin (3 mg/kg daily for 10 days, tapered over 1 month) has shown benefit in multiple studies with reduced mortality compared to predicted rates 3
• Systemic corticosteroids, particularly early methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 3, 4
• Intravenous immunoglobulin (IVIG) at 0.4 g/kg/day for 5 days may be considered as additional therapy with systemic steroids in severe cases 4

Infection Prevention and Management

• Monitor for signs of systemic infection rather than using prophylactic antibiotics, which may increase skin colonization 1, 2
• Take regular skin swabs for culture to detect predominant organisms 1
• Only institute antimicrobial therapy if there are clinical signs of infection 1, 2

Discharge Planning and Follow-up

• Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications 3
• Encourage patients to wear a MedicAlert bracelet or amulet bearing the name of the culprit drug 3
• Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care 3, 2
• Report the adverse drug reaction to pharmacovigilance authorities 3
• Arrange follow-up with dermatology and ophthalmology within weeks of discharge 2
• Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence 3
• Consider referral to support groups such as SJS Awareness U.K. 3

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality risk 1, 2
• 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 1, 2
• Failure to involve ophthalmology early can lead to permanent visual impairment 3, 1
• Neglecting to monitor for and treat sepsis, which is the most common cause of death in SJS/TEN 2, 5