What is the appropriate management of Stevens-Johnson syndrome?

Management of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit drugs and transfer patients with >10% body surface area epidermal detachment to a specialized burn unit or ICU within hours of diagnosis, as delayed transfer markedly increases mortality. 1, 2

Immediate Life-Saving Actions

• 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
• Arrange mandatory ophthalmology consultation within 24 hours of diagnosis; failure to do so is associated with permanent visual impairment 1, 2
• Record every medication taken in the preceding two months, including over-the-counter products, with exact start dates to identify the culprit drug 1
• Withdraw the suspected medication immediately, as this decreases the risk of death 1

Specialized Care Environment

• Admit to a burn center or ICU staffed by a multidisciplinary team including dermatology, intensive care, ophthalmology, and specialist 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
• Ensure daily bedside care is performed by nurses experienced in skin-fragility disorders 1, 2

Skin and Wound Management

Handle skin with extreme gentleness using anti-shear techniques to avoid further epidermal detachment. 1, 2

• Clean wounds gently 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 serve as a biological dressing 1, 2
• Decompress blisters by piercing and expressing or aspirating fluid 1
• Cover denuded dermis with non-adherent dressings (e.g., Mepitel or Telfa) and secondary foam or burn dressings to collect exudate 1, 2
• 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; they increase colonization with resistant organisms (especially Candida species) without improving outcomes. 1, 2

• 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 is evident 1, 2
• Consider herpes simplex virus reactivation in slowly healing eroded or vesicular areas, particularly 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
• 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 phase 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, 2

Urogenital Care

• Insert urinary catheter when urogenital involvement causes dysuria, retention, or when accurate output monitoring is required 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 for comfort at rest 1, 2
• Add opioid analgesia for breakthrough pain 1, 2

Systemic Immunomodulatory Therapy

Initiate intravenous methylprednisolone 0.5-1 mg/kg (or equivalent) within 72 hours of disease onset, then switch to oral corticosteroids with a taper of at least 4 weeks. 1, 2

• Consider cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month; studies show reduced mortality compared with predicted rates 1, 3
• Evidence for intravenous immunoglobulin (IVIg) is equivocal—pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 1
• Thalidomide was associated with excess deaths in one randomized trial and should be avoided 1

The controversy over systemic corticosteroids remains unresolved in older literature 4, but more recent guidelines favor early use within 72 hours 1, 2. The key is early initiation and appropriate tapering to balance immune suppression against infection risk.

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

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, 2
• Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists 1, 2
• Mortality in children is lower than adults (0-8.5% depending on severity), but recurrence occurs in up to 18% of cases 2

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 (MHRA Yellow Card Scheme in the U.K.) 1, 2
• Inform patients that fatigue and lethargy may persist for several weeks after discharge and that convalescence is required 1, 2
• Organize dermatology and ophthalmology outpatient appointments within a few weeks of discharge 1, 3
• Consider referral to support groups such as SJS Awareness U.K. 1

Critical Pitfalls to Avoid

• Delayed transfer to specialized care significantly increases mortality 1, 2
• Failure to involve ophthalmology within 24 hours leads to permanent visual impairment 1, 2
• Indiscriminate prophylactic antibiotics increase resistant organism colonization without benefit 1, 2
• Continued use of the culprit medication worsens the condition and increases mortality 1, 2
• Over-aggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal edema 1, 2
• Examining the patient without awareness of skin fragility can cause additional epidermal detachment; all clinicians must be warned of this risk 2