Treatment of Stevens-Johnson Syndrome
Stevens-Johnson syndrome requires immediate drug discontinuation, transfer to a specialized burn unit or ICU, and comprehensive multidisciplinary supportive care with early consideration of cyclosporine as the preferred systemic immunomodulatory therapy. 1, 2
Immediate Actions
Discontinue all suspected culprit drugs immediately – this is the single most critical intervention that directly impacts mortality. 2 Calculate SCORTEN on admission to predict mortality risk and guide intensity of care. 2
Transfer patients to specialized centers without delay, particularly when body surface area involvement exceeds 10%. 2 Optimal care requires burn units or ICUs with multidisciplinary teams including dermatology, intensive care, ophthalmology, and specialist nursing. 2 Early transfer reduces mortality; delays adversely affect outcomes. 2
Supportive Care Framework
Skin and Wound Management
Minimize shearing forces when handling skin to prevent further epidermal detachment. 2 Apply bland emollients frequently (white soft paraffin) to support barrier function and reduce transcutaneous water loss. 2
Leave detached epidermis in situ to act as a biological dressing. 2 Decompress blisters by piercing and expressing fluid. 2 Apply nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate. 2 Consider silver-containing products only for sloughy areas. 2
Cleanse wounds gently by irrigating with warmed sterile water, saline, or chlorhexidine (1:5000). 2
Fluid and Nutritional Management
Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload that leads to pulmonary, cutaneous, and intestinal edema. 2 Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes. 2
Deliver continuous enteral nutrition throughout the acute phase: 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery. 2 Consider nasogastric feeding when oral intake is precluded by buccal mucositis. 2
Pain Management
Provide adequate background simple analgesia to ensure comfort at rest, with addition of opiates as required delivered enterally, by patient-controlled analgesia, or via infusion. 1 If moderate-to-severe pain is uncontrolled by simple analgesia, initiate an opiate-based regimen using morphine. 1 Monitor level of consciousness, respiratory rate, and oxygen saturation carefully. 1
Infection Prevention
Do NOT use prophylactic antibiotics – they increase skin colonization with resistant organisms, particularly Candida. 2 Monitor for signs of infection rather than treating prophylactically. 2
Obtain regular skin swabs for culture to detect predominant organisms. 2 Institute targeted antimicrobial therapy only when clinical signs of infection appear. 2 Fever from SJS/TEN itself complicates detection of secondary sepsis, requiring careful monitoring. 2
Additional Supportive Medications
Administer low molecular weight heparin as prophylactic anticoagulation against venous thromboembolism in immobile patients. 1
Provide proton pump inhibitor during the acute phase for patients in whom enteral nutrition cannot be established, to protect against upper gastrointestinal stress ulceration. 1
Consider recombinant human G-CSF in neutropenic patients to resist infectious complications and potentially enhance re-epithelialization. 1
Mucosal Management
Ocular Care (Critical for Preventing Blindness)
Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews during the acute illness. 2 This is non-negotiable – 74% of patients develop acute ocular involvement, and 50% develop late complications including severe dry eyes and trichiasis. 1
Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness. 2 Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions. 2
Use topical antibiotics when corneal fluorescein staining or ulceration is present. 2 Consider topical corticosteroid drops under ophthalmologist supervision to reduce ocular surface damage. 2
Consider amniotic membrane transplantation (AMT) in the acute phase – retrospective studies demonstrate significantly better visual outcomes compared to medical management alone. 1, 2 Sutureless AMT using symblepharon rings shows better outcomes than sutured AMT. 1
Oral Care
Examine the mouth as part of initial assessment with daily oral review during acute illness. 1, 2 Apply white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness. 1, 2
Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating. 1, 2 For inadequate pain control, use topical anesthetic preparations such as viscous lidocaine 2% (15 mL per application) or cocaine mouthwashes 2-5% three times daily for severe oral discomfort. 1
Apply antiseptic oral rinse twice daily to reduce bacterial colonization: 1.5% hydrogen peroxide mouthwash or 0.2% chlorhexidine digluconate. 1 Protect ulcerated mucosal surfaces with mucoprotectant mouthwash three times daily. 1
Consider topical corticosteroids (betamethasone sodium phosphate 0.5 mg in 10 mL water as 3-minute rinse-and-spit, or clobetasol propionate 0.05% mixed with Orabase applied directly to affected mucosae). 1
Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week. 2 Monitor for HSV reactivation. 1
Urogenital Care
Examine the urogenital tract as part of initial assessment with daily documented review during acute illness. 1 In women, arrange early assessment by a vulval specialist for consideration of dilators to prevent vaginal synechiae. 1
Apply white soft paraffin ointment to urogenital skin and mucosae immediately, then every 4 hours through the acute illness. 1
Use Mepitel dressings to eroded areas in the vulva and vagina to reduce pain and prevent adhesions. 1 Insert a dilator or tampon wrapped in Mepitel into the vagina to prevent formation of synechiae. 1
Catheterize all patients to prevent strictures forming in the urethra. 1 Consider applying potent topical corticosteroid ointment once daily to involved, noneroded urogenital surfaces. 1
Systemic Immunomodulatory Therapy
Cyclosporine (3 mg/kg daily for 10 days, tapered over 1 month) is the preferred systemic therapy – multiple studies demonstrate reduced mortality compared to predicted rates. 2, 3, 4 There is increasing evidence that cyclosporine and TNF-α inhibitors decrease mortality. 3
Systemic corticosteroids, particularly early IV methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset. 2, 3 However, efficacy remains uncertain and controversial. 3, 5 While many authors agree corticosteroids suppress progression, they greatly enhance infection risk. 5 If used, administer only during the progression phase and withdraw as soon as possible. 5
The evidence for intravenous immunoglobulins remains limited to small series and requires confirmation by large clinical trials. 5
Discharge Planning and Long-Term Management
Provide written information about the culprit drug(s) to avoid and any potentially cross-reactive medications. 2 Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug. 2
Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care. 2 Report the adverse drug reaction to pharmacovigilance authorities. 2
Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence. 2 Consider referral to support groups. 2
Common Pitfalls to Avoid
Delayed recognition and transfer to specialized care significantly increases mortality. 2 This is the most critical error.
Indiscriminate prophylactic antibiotics increase resistant organism colonization, particularly Candida. 2
Overaggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema. 2
Failure to involve ophthalmology early leads to permanent ocular sequelae including blindness. 2 74% of patients develop acute ocular involvement. 1
Continued use of the culprit medication worsens the condition and increases mortality. 2
Failure to prevent vaginal synechiae in women through early vulval specialist involvement and use of dilators. 1