What is the recommended treatment for Stevens‑Johnson syndrome?

Treatment of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit medications and transfer patients with >10% body surface area epidermal detachment to a specialized burn center or ICU within hours of diagnosis—this is the single most critical intervention that directly impacts survival. 1, 2

Immediate Life-Saving Actions

• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores 0-7 correspond to mortality rates of approximately 1-99%) and guide intensity of care 3, 1, 2
• Arrange mandatory ophthalmology consultation within 24 hours of diagnosis, as failure to do so is associated with permanent visual impairment 1, 2
• Document all medications taken in the preceding 2 months, including over-the-counter and herbal products, with exact start dates 3, 1
• Common culprit drugs include allopurinol, carbamazepine, lamotrigine, nevirapine, oxicam NSAIDs, phenobarbital, phenytoin, sulfamethoxazole, and sulfasalazine 3

Specialized Care Environment

• Admit patients to a burn center or ICU staffed by a multidisciplinary team including dermatology, intensive care, ophthalmology, and specialist skincare nursing 3, 1, 2
• Provide barrier nursing in a temperature-controlled room (25-28°C) on a pressure-relieving mattress to prevent hypothermia and reduce infection risk 1, 2
• Early transfer to specialized centers reduces mortality; delayed transfer significantly increases death rates 3, 1, 2

Fluid and Nutritional Management

• Establish adequate intravenous fluid replacement guided by urine output and hemodynamic parameters, but avoid overaggressive resuscitation that causes pulmonary, cutaneous, and intestinal edema 1, 2
• Monitor fluid balance regularly using vital signs, urine output, and electrolyte measurements 1
• Provide continuous enteral nutrition delivering 20-25 kcal/kg/day during the catabolic phase, increasing to 25-30 kcal/kg/day during recovery 1
• Use nasogastric feeding when oral intake is impossible due to buccal mucositis 1

Skin and Wound Care

• Handle skin with extreme gentleness using anti-shear techniques to minimize further epidermal detachment 1, 2
• Leave detached epidermis in situ to act as a biological dressing—do not debride intact blisters 1, 2
• Cleanse wounds gently by irrigating with warmed sterile water, saline, or dilute chlorhexidine (1:5000) 1
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis, including denuded areas, every 2-4 hours 1, 2
• Cover denuded dermis with non-adherent dressings (e.g., Mepitel or Telfa) and secondary foam or burn dressings to collect exudate 1
• Apply silver-containing dressings only to sloughy areas 1
• Reserve surgical debridement with biosynthetic xenograft or allograft for clinical deterioration, extension of detachment, local sepsis, delayed healing, or wound conversion 1

Infection Prevention and Management

Do not use prophylactic systemic antibiotics—indiscriminate administration increases skin colonization with resistant organisms, particularly Candida, without improving outcomes. 1, 2

• Obtain bacterial and candidal swabs from three lesional sites on alternate days for culture 1, 2
• Monitor for clinical infection signs: confusion, hypotension, oliguria, desaturation, increased skin pain, rising C-reactive protein, and neutrophilia 1
• Initiate targeted antimicrobial therapy only when clinical signs of infection are present 1, 2
• Consider herpes simplex virus reactivation in slowly healing eroded or vesicular areas, particularly genital and oral sites 1

Mucosal Management

Ophthalmologic Care

• Perform ophthalmology examination within 24 hours of diagnosis and repeat daily throughout the acute phase 1, 2
• Apply preservative-free lubricating eye drops every 2 hours during the acute illness 1, 2
• Conduct daily ocular hygiene by an ophthalmologist or trained nurse to remove debris and break conjunctival adhesions 1
• Use topical antibiotics when corneal fluorescein staining or ulceration is present 1
• Apply topical corticosteroid eye drops under ophthalmology supervision to reduce ocular surface damage 1

Oral Care

• Apply white soft paraffin ointment to the lips immediately and then every 2 hours throughout the acute phase 1, 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, especially before meals 1
• Perform antiseptic oral rinse twice daily to lower bacterial colonization 1
• Provide topical anesthetic (viscous lidocaine 2% or cocaine mouthwash 2-5%) for severe oral discomfort 1
• Apply topical corticosteroids to oral mucosa as needed 1

Urogenital Care

• Insert urinary catheter when urogenital involvement causes dysuria, retention, or when accurate output monitoring is required 1
• Apply white soft paraffin ointment to urogenital skin and mucosa every 4 hours 1
• Use vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Pain Management

• Provide baseline non-opioid analgesia for comfort at rest 1
• Add opioid analgesia for breakthrough pain 1
• Consider patient-controlled analgesia where appropriate 4
• Consider sedation or general anesthesia for patient handling, repositioning, and dressing changes 4

Systemic Immunomodulatory Therapy

Cyclosporine 3 mg/kg daily for 10 days (tapered over 1 month) has shown the most consistent benefit with reduced mortality compared to predicted rates in multiple studies. 1, 2

• If using corticosteroids, initiate intravenous methylprednisolone 0.5-1 mg/kg (or equivalent) within 72 hours of disease onset, then switch to oral corticosteroids with a taper lasting at least 4 weeks 1, 2
• For immune-checkpoint-inhibitor-induced SJS/TEN, use methylprednisolone 1-2 mg/kg for grade 4 disease or 0.5-1 mg/kg for grade 3 disease 1
• Evidence for IVIG is equivocal—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 1, 5
• Thalidomide should be avoided as it was associated with excess deaths in a randomized trial 1, 6

Additional Supportive Measures

• Administer low-molecular-weight heparin as prophylactic anticoagulation for immobile patients 1
• Provide proton-pump inhibitor when enteral nutrition cannot be established 1
• Consider recombinant human G-CSF for neutropenic patients 1

Discharge Planning and Follow-Up

• Provide written information about the culprit drug(s) and any cross-reactive medications to avoid 1, 2
• Advise patients to wear a MedicAlert bracelet naming the culprit drug 1, 2
• Document the drug allergy in the medical record and inform all healthcare providers 1, 2
• Report the adverse drug reaction to national pharmacovigilance authorities 1, 2
• Inform patients that fatigue and lethargy may persist for several weeks after discharge 1
• Schedule ophthalmology follow-up to monitor for chronic ocular complications, as 50-63% develop late complications including severe dry eyes and trichiasis 2

Critical Pitfalls to Avoid

• Delayed transfer to specialized care significantly increases mortality 1, 2
• Continued use of the culprit medication worsens the condition and increases mortality 1
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment 1
• Overaggressive fluid resuscitation causes pulmonary, cutaneous, and intestinal edema 1
• Indiscriminate prophylactic antibiotics increase resistant organism colonization without benefit 1, 2