What is the appropriate acute management of Stevens-Johnson syndrome, including drug discontinuation, supportive care, and systemic therapy options?

Acute Management of Stevens-Johnson Syndrome

Immediate Life-Saving Actions

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 significantly increases mortality. 1, 2

• Calculate SCORTEN within the first 24 hours to predict mortality risk (scores 0-7 correspond to 1-99% mortality) and guide intensity of care 1, 2, 3
• Arrange mandatory ophthalmology consultation within 24 hours of diagnosis, as failure to do so leads to permanent visual impairment 1, 2, 3
• Document all medications taken in the previous 2 months, including over-the-counter products, with exact start dates 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

• 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
• Daily bedside care must be performed by specialist nurses experienced with skin-fragility disorders 1, 2

Skin and Wound Management

Handle skin with extreme gentleness using antishear techniques to minimize further epidermal detachment. 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 + 50% liquid paraffin) over the entire epidermis, including denuded areas, every 2-4 hours 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
• Use non-adherent dressings (e.g., Mepitel or Telfa) over denuded dermis, covered with 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, as they increase colonization with resistant organisms (particularly Candida albicans) without improving outcomes. 1, 2, 3

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

Fluid, Electrolyte, and Nutritional Support

Avoid over-aggressive fluid resuscitation, which causes pulmonary, cutaneous, and intestinal edema. 1, 2

• Monitor fluid balance regularly using vital signs, urine output, and electrolyte measurements 1, 2
• Provide continuous enteral nutrition: 20-25 kcal/kg/day during the catabolic phase, increasing to 25-30 kcal/kg/day during recovery 1, 2, 3
• Use nasogastric feeding when oral intake is impossible due to buccal mucositis 1, 2

Mucosal Management

Ophthalmologic Care

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 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 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, 2
• Perform antiseptic oral rinse twice daily to lower bacterial colonization 1, 2
• Provide topical anesthetic (viscous lidocaine 2% or cocaine mouthwash 2-5%) for severe oral discomfort 1, 2
• 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, 2

Pain Management

Provide baseline non-opioid analgesia to ensure comfort at rest, with additional opioid analgesia for breakthrough pain. 1, 2

Systemic Immunomodulatory Therapy

Start 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, 3

• Consider cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, as studies show reduced mortality compared with predicted rates 1, 2, 3
• High-strength topical corticosteroids may be applied to affected skin areas 2

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients 1, 3
• Provide proton pump inhibitor if enteral nutrition cannot be established 1, 3

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 and that convalescence is required 1, 2
• Arrange dermatology follow-up within weeks of discharge 3
• Schedule ophthalmology follow-up to monitor for chronic ocular complications 3

Critical Pitfalls to Avoid

• Delayed transfer to specialized care significantly increases mortality 1, 2, 3
• Examining the patient without awareness of skin fragility causes additional epidermal detachment; all clinicians must be warned of this risk 2
• Continued use of the culprit medication worsens the condition and raises mortality 2
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment 1, 2, 3

Special Pediatric Considerations

Infectious triggers account for up to 50% of pediatric SJS/TEN; test for Mycoplasma pneumoniae and herpes simplex virus and involve infectious disease specialists. 2, 4

• Mortality in children is lower than in adults (0-8.5% depending on severity), but recurrence occurs in up to 18% of cases 2
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 2