Management of Stevens-Johnson Syndrome
Immediately discontinue all suspected culprit drugs and transfer the patient to a specialized burn unit or intensive care unit with multidisciplinary expertise in managing SJS/TEN. 1
Initial Assessment and Stabilization
Calculate SCORTEN within 24 hours of admission to predict mortality risk and guide intensity of care. 1, 2, 3 Transfer patients with >10% body surface area (BSA) epidermal detachment to a burn center or ICU with experience managing extensive skin loss. 1, 2, 3 For children, transfer is particularly important when BSA detachment exceeds 10%, or if comorbidities exist, or ventilation is required. 1
Establish intravenous access and initiate careful fluid resuscitation guided by urine output, avoiding overaggressive hydration that can cause pulmonary, cutaneous, and intestinal edema. 2, 3 Catheterize patients to monitor urine output and prevent urethral strictures. 1, 3
Supportive Care: The Foundation of Treatment
Wound Management
Handle skin with extreme gentleness to minimize shearing forces that cause further epidermal detachment. 2, 3 Leave detached epidermis in situ to act as a biological dressing, decompressing blisters by piercing and expressing fluid. 2
Cleanse wounds by gently irrigating with warmed sterile water, saline, or dilute chlorhexidine (1:5000). 2, 3 Apply greasy emollients (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis every 4 hours, including denuded areas. 1, 2, 3
Apply nonadherent dressings (Mepitel or Telfa) to exposed dermis with secondary foam or burn dressings to collect exudate, reduce fluid and protein loss, limit microbial colonization, control pain, and accelerate re-epithelialization. 2, 3 Consider silver-containing dressings only for sloughy areas. 2
Environmental Control
Nurse patients in a temperature-controlled room (25-28°C) on a pressure-relieving mattress with controlled humidity. 1, 3 This reduces metabolic demands and transcutaneous water loss. 2
Pain Management
Provide adequate background analgesia with simple analgesics, supplemented by intravenous opioid infusions or patient-controlled analgesia for breakthrough pain. 2, 3 Consider sedation or general anesthesia for dressing changes and patient repositioning. 3
Nutritional Support
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 Use nasogastric feeding when oral intake is precluded by buccal mucositis. 2
Infection Prevention and Management
Do not use prophylactic systemic antibiotics, as this increases skin colonization, particularly with Candida albicans and resistant organisms. 2, 3 This is a critical pitfall to avoid. 2, 3
Take regular skin swabs for bacterial and candidal culture from lesional skin, particularly sloughy areas. 2, 3 Monitor for clinical signs of systemic infection including confusion, hypotension, reduced urine output, and reduced oxygen saturation. 3
Institute targeted antimicrobial therapy only when clinical signs of infection are present or when monoculture of organisms appears on swabs from multiple sites, indicating increased likelihood of invasive infection. 2, 3 Sepsis is the most common cause of death in SJS/TEN. 3, 4
Mucosal Management
Ophthalmologic Care (Critical Priority)
Arrange ophthalmology consultation within 24 hours of diagnosis and continue daily reviews throughout the acute illness. 1, 2, 3 Failure to provide early eye care can result in permanent visual impairment. 3
Apply preservative-free lubricant eye drops every 2 hours and lubricating ointments throughout the acute illness. 2 Perform daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and lyse conjunctival adhesions to prevent symblepharon formation. 1, 2
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. 1, 2
Oral Care
Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating. 1, 2 For inadequate pain control, apply topical anesthetics such as viscous lidocaine 2% (15 mL per application) or cocaine mouthwashes 2-5% three times daily for severe discomfort. 1, 2
Apply antiseptic oral rinse twice daily (1.5% hydrogen peroxide or 0.2% chlorhexidine digluconate, diluted up to 50% to reduce soreness). 1 Clean the mouth daily with warm saline mouthwashes or oral sponge, sweeping gently in labial and buccal sulci to reduce risk of fibrotic scars. 1
Consider topical corticosteroids four times daily (betamethasone sodium phosphate 0.5 mg in 10 mL water as rinse-and-spit, or clobetasol propionate 0.05% mixed with Orabase applied directly to sulci). 1, 2
Monitor for and treat secondary infections: use nystatin oral suspension 100,000 units four times daily or miconazole oral gel for candidal infection. 1 Consider HSV reactivation if oral mucosa shows slow healing. 1
Urogenital Care
Examine the urogenital tract as part of initial assessment, with early assessment by a vulval specialist in women for consideration of dilators to prevent vaginal synechiae. 1 Perform daily documented urogenital review during acute illness. 1
Apply white soft paraffin ointment to urogenital skin and mucosae immediately and every 4 hours throughout acute illness. 1, 2 Use Mepitel dressings on eroded vulval and vaginal areas to reduce pain and prevent adhesions. 1, 2 Insert a dilator or tampon wrapped in Mepitel into the vagina to prevent synechiae formation. 1, 2
Consider applying potent topical corticosteroid ointment once daily to involved, noneroded urogenital surfaces. 1 Check uncircumcised males for preputial retractability. 1
Systemic Immunomodulatory Therapy
Ciclosporin (Cyclosporine)
Ciclosporin at 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, 4, 5 This represents the strongest evidence for systemic therapy. 2
Systemic Corticosteroids
Systemic corticosteroids, particularly early intravenous methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset. 2, 3 However, evidence remains controversial regarding their use, as they may increase infection risk. 4, 6 If used, administer only during the progression phase and withdraw as soon as possible. 6
Other Therapies
Evidence for intravenous immunoglobulin (IVIg) and TNF-α inhibitors exists but is less robust than for ciclosporin. 4, 5, 6 No controlled clinical trials have definitively established optimal systemic therapy. 1, 6
Multidisciplinary Team Approach
Coordinate care through a multidisciplinary team led by a specialist in skin failure (dermatology or plastic surgery), including intensive care, ophthalmology, and specialist skincare nursing. 1, 2, 3 Additional specialists may include respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy. 3
Discharge Planning and Follow-up
Provide written information about culprit drug(s) to avoid and any potentially cross-reactive medications. 2, 3 Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug. 1, 2, 3
Document the drug allergy in the patient's medical records and inform all healthcare providers involved in their care. 2, 3 Report the adverse drug reaction to pharmacovigilance authorities. 2, 3
Inform patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence. 2 Arrange follow-up with dermatology and ophthalmology within weeks of discharge. 3 Consider referral to support groups such as SJS Awareness U.K. 2
Special Considerations for Children
Recurrence is more common in children (up to 18% of cases), often because the precipitant is usually infection rather than drugs. 1 Mortality appears lower in children than adults, making management of long-term sequelae particularly important in the pediatric population. 1 Consider telemedicine advice from a specialist center for pediatric cases. 1
Critical Pitfalls to Avoid
- Delayed recognition and transfer significantly increases mortality risk 2, 7
- Indiscriminate prophylactic antibiotics increase resistant organism colonization 2, 3
- Overaggressive fluid resuscitation causes pulmonary and tissue edema 2, 3
- Failure to involve ophthalmology early leads to permanent visual sequelae 2, 3
- Continued use of culprit medication worsens condition and increases mortality 2, 3
- Neglecting mucosal care results in long-term complications including blindness and strictures 3, 4, 6