What is the treatment for Steven Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit medications and transfer the patient to a specialized burn unit or ICU with multidisciplinary SJS/TEN experience, particularly when body surface area involvement exceeds 10%. 1, 2

Initial Assessment and Triage

• Calculate SCORTEN on admission to predict mortality risk and guide intensity of care 1, 2
• Obtain skin biopsy to confirm diagnosis, looking for confluent epidermal necrosis with subepidermal vesicle formation 2
• Transfer without delay to centers with multidisciplinary teams including dermatology, intensive care, burn surgery, and ophthalmology—early transfer reduces mortality 2, 3
• Barrier nurse in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 1, 2

Supportive Care Framework

Fluid Management

• Establish adequate intravenous fluid replacement guided by urine output and end organ function markers 1
• Fluid requirements are lower than burn formulas predict—overaggressive resuscitation causes pulmonary, cutaneous, and intestinal edema 1, 2
• Monitor fluid balance carefully with catheterization if clinically indicated 1
• Use continuous invasive hemodynamic monitoring through central or arterial lines in severely affected cases, with serial lactate, base deficit, and electrolyte measurements 1

Skin and Wound Care

• Handle skin carefully to minimize shearing forces and prevent further epidermal detachment 1
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000) 1
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis, including denuded areas 1, 2
• Leave detached epidermis in situ to act as a biological dressing; decompress blisters by piercing and expression of fluid 1, 3
• Apply nonadherent dressings (Mepitel or Telfa) to denuded dermis with secondary foam or burn dressings (Exu-Dry) to collect exudate 1, 2
• Limit epidermal trauma by avoiding adhesive dressings, ECG leads, blood pressure cuffs, and identification tags—use soft silicone tapes for essential items 1

Infection Prevention and Management

• Do not use prophylactic antibiotics—they increase skin colonization with resistant organisms, particularly Candida 1, 2
• Take swabs for bacterial and candidal culture from lesional skin throughout the acute phase 1
• Institute targeted antimicrobial therapy only when clinical signs of infection appear 1, 2
• Apply topical antimicrobial agents to sloughy areas only, with choice guided by local microbiological advice 1

Nutrition

• Provide continuous enteral nutrition throughout the acute phase, either orally or via nasogastric feeding with silicone tube if buccal mucositis precludes oral intake 1
• Deliver 20-25 kcal/kg daily during the early catabolic phase 1, 2
• Increase to 25-30 kcal/kg daily during the anabolic recovery phase 1, 2

Analgesia

• Use appropriate validated pain tools to assess pain at least once daily 1
• Provide adequate background simple analgesia with additional opioid analgesia for breakthrough pain 1, 2
• Administer intravenous opioid infusions in those not tolerating oral medication, or patient-controlled analgesia where appropriate 1
• Consider sedation or general anesthesia for pain associated with dressing changes and repositioning 1

Mucosal Management

Ocular Care

• Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews during acute illness—failure to involve ophthalmology early leads to permanent sequelae 1, 2, 3
• Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness 2, 3
• Perform daily ocular hygiene by ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 2, 3
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1, 2
• Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage 2
• Consider amniotic membrane transplantation in the acute phase for severe cases 2

Oral Care

• Examine mouth as part of initial assessment with daily oral review during acute illness 2, 3
• Apply white soft paraffin ointment to lips immediately, then every 2 hours throughout acute illness 2, 3
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 1, 2, 3
• Use antiseptic oral rinse containing chlorhexidine twice daily 2, 3
• Clean mouth daily with warm saline mouthwashes or oral sponge 3
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 2

Urogenital Care

• Perform daily urogenital review during acute illness 2, 3
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 2, 3
• Use potent topical corticosteroid ointment once daily to involved, noneroded surfaces 3
• Apply silicone dressings (Mepitel) to eroded areas 3
• Consider urinary catheterization when urogenital involvement causes dysuria or retention 2, 3
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Systemic Therapy Options

The evidence for systemic immunomodulation remains controversial, but early treatment within 72 hours appears most beneficial. 2, 3

• Cyclosporine (3 mg/kg daily for 10 days, tapered over 1 month) has shown benefit in multiple studies with reduced mortality compared to predicted rates 2, 3
• Systemic corticosteroids, particularly early methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 2, 3
• A recent large US multicenter study found lowest mortality among patients receiving both corticosteroids and intravenous immunoglobulin (standardized mortality ratio 0.52) 4
• Consider recombinant human G-CSF in neutropenic patients 1

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation against venous thromboembolism in immobile patients 1
• Consider proton pump inhibitor during acute phase in patients where enteral nutrition cannot be established 1
• Assess respiratory symptoms and hypoxemia on admission—perform fiberoptic bronchoscopy to assess airway involvement and discuss early with intensivist 3

Discharge Planning and Follow-up

• Provide written information about culprit drug(s) to avoid and potentially cross-reactive medications 2, 3
• Encourage patient to wear MedicAlert bracelet bearing the name of the culprit drug 2, 3
• Document drug allergy in patient's medical records and inform all healthcare providers involved in care 2, 3
• Report adverse drug reaction to national pharmacovigilance authorities 2, 3
• Organize dermatology outpatient appointment and, if required, ophthalmology appointment within a few weeks of discharge 3
• Inform patients about potential fatigue and lethargy for several weeks following discharge 2

Common Pitfalls to Avoid

• Delayed recognition and transfer to specialized care significantly increases mortality—early transfer is critical 2, 5
• Continued use of culprit medication worsens condition and increases mortality 2, 3
• Indiscriminate prophylactic antibiotics increase resistant organism colonization 1, 2
• Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2
• Failure to involve ophthalmology within 24 hours results in permanent ocular sequelae 1, 2