What is the appropriate management of Stevens‑Johnson syndrome?

Management 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, as delayed transfer markedly increases mortality risk. 1, 2

Initial Assessment and Risk Stratification

• Calculate the SCORTEN score within the first 24 hours to predict mortality—scores range from 0 to 7, corresponding to mortality rates from 1% to 99%—and use this to guide intensity of care 1, 3
• Document all medications taken in the preceding 2 months, including over-the-counter and herbal products, with exact start dates to identify the culprit drug 4, 3
• Obtain skin biopsy from lesional skin adjacent to a blister showing confluent epidermal necrosis with subepidermal vesicle formation 3
• Order baseline investigations: complete blood count, electrolytes, liver and renal function, glucose, coagulation studies, mycoplasma serology (especially in children and young adults), and chest X-ray 1, 3

Specialized Care Environment

• Admit to a burn center or ICU with a multidisciplinary team including dermatology, intensive care, ophthalmology, and specialist skincare nursing 1, 2, 3
• Provide barrier nursing in a temperature-controlled room at 25-28°C on a pressure-relieving mattress to prevent hypothermia and reduce infection risk 1, 2
• Early transfer to specialized centers reduces mortality; any delay adversely affects outcomes 1

Skin and Wound Management

• Handle skin with extreme gentleness using antishear techniques to minimize further epidermal detachment—all clinicians must be warned that examining the patient without awareness of skin fragility can cause additional damage 1, 2
• Cleanse wounds gently by irrigating with warmed sterile water, saline, or dilute chlorhexidine (1:5000) 1, 2
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis, including denuded areas, every 2-4 hours to support barrier function and reduce trans-epidermal water loss 1, 2
• Leave detached epidermis in situ to act as a biological dressing 1, 2
• Decompress blisters by piercing and expressing or aspirating fluid 1
• Apply non-adherent dressings (e.g., Mepitel or Telfa) over denuded dermis, covered with secondary foam or burn dressings to collect exudate 1, 2
• Reserve silver-containing dressings only for sloughy areas 1
• Daily bedside care should be performed by specialist nurses experienced with skin-fragility disorders 1

Infection Prevention and Management

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

• Obtain bacterial and candidal swabs from three lesional sites on alternate days for culture 1
• Monitor closely for 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, especially genital and oral sites 1

Ophthalmologic Management

Arrange ophthalmology consultation within 24 hours of diagnosis—failure to do so is associated with permanent visual impairment—and continue daily examinations throughout the acute phase. 1, 2, 3

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

Oral and Mucosal Care

• Apply white soft paraffin ointment to the lips immediately and then every 2 hours throughout the acute phase 1, 2
• Use an anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, especially before meals 1, 2
• Perform an 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 a urinary catheter when urogenital involvement causes dysuria, retention, or to monitor output 1, 2
• Apply white soft paraffin ointment to urogenital skin and mucosa every 4 hours 1, 2
• Use vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 1

Fluid, Electrolyte, and Nutritional Support

• Avoid over-aggressive fluid resuscitation, which causes pulmonary, cutaneous, and intestinal edema 1, 3
• Monitor fluid balance regularly using vital signs, urine output, and electrolyte measurements 1
• Provide continuous enteral nutrition: 20-25 kcal/kg/day during the catabolic phase, increasing to 25-30 kcal/kg/day during recovery 1, 3
• Use nasogastric feeding when oral intake is impossible due to buccal mucositis 1

Pain Management

• Provide baseline non-opioid analgesia to ensure comfort at rest 1
• Add opioid analgesia for breakthrough pain 1

Systemic Immunomodulatory Therapy

Administer 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, 3

• Early methylprednisolone pulse therapy may be beneficial if initiated within 72 hours 1, 2
• Consider cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month—studies show reduced mortality compared with predicted SCORTEN rates 1, 3
• For immune-checkpoint-inhibitor-induced SJS/TEN: use methylprednisolone 0.5-1 mg/kg for grade 3 disease or 1-2 mg/kg for grade 4 disease, and permanently discontinue the offending agent 1

The evidence regarding intravenous immunoglobulin (IVIG) is equivocal—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75)—so it should be reserved for patients with severe disease who do not respond to corticosteroids 1. Thalidomide was associated with excess deaths in one randomized trial and should be avoided 1.

Special Considerations for Pediatric Patients

• Infectious triggers account for up to 50% of pediatric SJS/TEN—test for Mycoplasma pneumoniae and herpes simplex virus and involve infectious disease specialists 1
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 1
• Mortality in children is lower than in adults (0-8.5% depending on severity), but recurrence occurs in up to 18% of cases 1

Airway Management

• Respiratory symptoms and hypoxemia on admission should prompt urgent discussion with an intensivist and rapid transfer to ICU or burn center 1, 2
• Perform fibreoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium 1

Discharge Planning and Follow-Up

• Provide written information about the identified culprit drug(s) and any cross-reactive agents to avoid permanently 1, 2, 3
• Advise patients to wear a MedicAlert bracelet naming the culprit drug 1, 2, 3
• Document the drug allergy in the medical record and inform all healthcare providers 1, 2, 3
• Report the adverse drug reaction to national pharmacovigilance authorities 1, 2, 3
• Counsel patients that fatigue and lethargy may persist for several weeks after discharge and that convalescence is required 1, 3
• Offer referral to support groups such as SJS Awareness U.K. 1
• Organize dermatology and ophthalmology outpatient appointments within a few weeks of discharge 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 raises mortality 1
• Over-aggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 3
• Indiscriminate prophylactic antibiotics increase resistant organism colonization without improving outcomes 1, 2
• Failure to involve ophthalmology within 24 hours can lead to permanent visual sequelae 1, 2, 3