What is the management and treatment approach for a patient suspected of having Steven Johnson syndrome?

Management of Stevens-Johnson Syndrome

Immediately discontinue all suspected culprit drugs and transfer patients with >10% body surface area (BSA) epidermal detachment to a specialized burn center or ICU without delay, as this is the single most critical intervention to reduce mortality. 1

Immediate Recognition and Triage

• Calculate SCORTEN within 24 hours to predict mortality risk (scores 0-7 correlate with mortality from 1% to 99%) and guide intensity of care 1, 2
• Look for painful mucocutaneous eruptions with targetoid lesions, blistering, and epidermal detachment involving skin, oral mucosa, eyes, and genitalia 3, 4
• Document all medications taken in the previous 2 months, including over-the-counter and herbal products, with exact start dates 2, 5
• Obtain skin biopsy showing confluent epidermal necrosis with subepidermal vesicle formation to confirm diagnosis 2

Critical pitfall: Delayed transfer to specialized centers significantly increases mortality; transfer must occur within hours, not days 1, 5

Specialized Care Environment

• Admit to burn center or ICU with multidisciplinary team including dermatology/plastic surgery, intensive care, ophthalmology, and specialist skincare nursing 1
• Barrier-nurse in temperature-controlled side room (25-28°C) with humidity control on pressure-relieving mattress 1
• Rapid admission to burn centers demonstrates improved survival in systematic reviews 1

Supportive Care Protocol

Fluid Management

• Establish IV access and initiate fluid resuscitation guided by urine output and hemodynamic parameters 2, 5
• Avoid overaggressive replacement which causes pulmonary, cutaneous, and intestinal edema 2, 5
• Monitor with urinary catheterization when clinically indicated 2, 5

Skin Management

• Handle skin with extreme care to minimize shearing forces that cause further epidermal detachment 1
• Leave detached epidermis in situ to act as biological dressing 1, 2
• Irrigate wounds gently with warmed sterile water, saline, or chlorhexidine (1/5000) 2, 5
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis including denuded areas every few hours 1, 2
• Cover denuded dermis with nonadherent dressings (Mepitel, Telfa) plus secondary foam dressings to collect exudate 1, 5
• Consider surgical debridement with biosynthetic xenograft or allograft only if conservative management fails with clinical deterioration or local sepsis 1

Infection Prevention and Management

• Do NOT use prophylactic antibiotics as this increases skin colonization with resistant organisms, particularly Candida albicans 1, 2, 5
• Take swabs for bacterial and candidal culture from three lesional areas on alternate days 2, 5
• Institute targeted antimicrobial therapy only when clinical signs of infection appear (confusion, hypotension, reduced urine output, reduced oxygen saturation, increased skin pain) 1, 2
• Monitor C-reactive protein and neutrophilia as indicators of sepsis 2

Critical pitfall: Sepsis is the most common cause of death in SJS/TEN; fever from the disease itself complicates detection of secondary infection 1, 5

Nutrition Support

• Provide continuous enteral nutrition throughout acute phase: 20-25 kcal/kg daily during catabolic phase, increasing to 25-30 kcal/kg during recovery 2
• Use nasogastric feeding when oral intake precluded by buccal mucositis 2

Pain Management

• Use validated pain assessment tools at least once daily 2, 5
• Provide adequate background analgesia (simple analgesics plus opioids) to ensure comfort at rest 1, 2
• Consider patient-controlled analgesia or sedation/general anesthesia for dressing changes 5

Mucosal Management

Ocular Care (Highest Priority)

• Arrange ophthalmology consultation within 24 hours with daily examinations throughout acute phase 1, 2, 5
• Apply preservative-free lubricant eye drops every 2 hours throughout acute illness 2, 5
• Perform daily ocular hygiene by ophthalmologist or trained nurse to remove inflammatory debris and break down conjunctival adhesions 2
• Use topical antibiotics when corneal fluorescein staining or ulceration present 2
• Consider amniotic membrane transplantation in acute phase for significantly better visual outcomes 2

Critical pitfall: Neglecting eye care results in permanent visual impairment; ocular sequelae are among the most common long-term complications 2, 5

Oral Care

• Perform daily oral review during acute illness 2
• Apply white soft paraffin ointment to lips every 2 hours 2
• Use anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 2
• Use antiseptic oral rinse twice daily 2
• Treat candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week 2

Urogenital Care

• Perform regular examination of urogenital tract during acute illness 2
• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 2
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 2

Systemic Immunomodulatory Therapy

The evidence for systemic therapy remains equivocal, but early intervention within 72 hours may provide benefit:

• Cyclosporine (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
• Systemic corticosteroids (particularly early IV methylprednisolone pulse therapy) may be beneficial if started within 72 hours of onset 2, 6, 7, 8
• IVIg has equivocal evidence: pooled analysis showed no mortality benefit (OR 1.00,95% CI 0.58-1.75) 2
• Avoid thalidomide: associated with excess deaths in randomized trial 2

The FDA label indicates corticosteroids are approved for severe erythema multiforme (Stevens-Johnson syndrome) 6

Airway and Respiratory Management

• Respiratory symptoms and hypoxemia on admission require urgent intensivist discussion and rapid ICU/burn center transfer 2
• Consider fibreoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, and mechanically remove sloughed bronchial epithelium 2

Additional Supportive Measures

• Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients 2
• Provide proton pump inhibitor if enteral nutrition cannot be established 2
• Consider recombinant human G-CSF for neutropenic patients 2

Discharge Planning and Follow-up

• Provide written information about culprit drug(s) to avoid and potentially cross-reactive medications 2
• Encourage wearing MedicAlert bracelet bearing the name of the culprit drug 2
• Document drug allergy in patient's medical records and inform all healthcare providers involved in care 2
• Report adverse drug reaction to national pharmacovigilance authorities 2
• Arrange follow-up with dermatology and ophthalmology within weeks of discharge 5
• Inform patients about potential fatigue and lethargy for several weeks following discharge 2