What is the management approach for a patient with Steven's Johnson syndrome (Toxic Epidermal Necrolysis), considering their recent medical history and potential triggers?

Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

Immediately discontinue all suspected culprit drugs and transfer patients with >10% body surface area (BSA) involvement to a burn center or specialized ICU without delay, as rapid admission to these facilities is associated with improved survival. 1, 2

Initial Assessment and Immediate Actions

Upon suspicion of SJS/TEN, three critical steps must occur simultaneously:

• Calculate SCORTEN within the first 24 hours to predict mortality risk (ranging from 1% with 0 parameters to 99% with 7 parameters) and guide management intensity 1, 2
• Stop all potential culprit drugs immediately - this is the single most important intervention to halt disease progression 2, 3
• Refer immediately to a multidisciplinary team coordinated by dermatology and/or plastic surgery specialists 1

Obtain bacterial swabs from lesional skin for culture and sensitivity, and perform skin biopsy for histopathological confirmation 2.

Care Setting and Environment

Patients with >10% BSA epidermal loss require admission to a burn center or ICU with specific SJS/TEN experience - delayed transfer is associated with increased mortality 1, 2. Three studies and a systematic review have demonstrated that rapid admission to burn centers improves survival 1.

Environmental requirements include:

• Barrier-nursing in a side room with controlled humidity 1, 2
• Pressure-relieving mattress to prevent further skin damage 1
• Ambient temperature maintained between 25°C and 28°C to prevent hypothermia from impaired thermoregulation 1, 2

Multidisciplinary Team Composition

The MDT must include 1:

• Dermatology and/or plastic surgery (team coordinator)
• Intensive care specialists
• Ophthalmology (mandatory - ocular involvement occurs in most cases)
• Specialist skincare nursing
• Additional consultants: respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, and pharmacy

Skin and Wound Management

Conservative Approach (First-Line)

Minimize shearing forces during all patient handling and positioning - this is critical as necrotic epidermis readily detaches from underlying dermis 1, 2. Day-to-day care should be performed by specialist nurses familiar with skin fragility disorders 1.

Specific wound care includes:

• Leave detached epidermis in situ to act as a biological dressing 2
• Apply greasy emollient frequently over the entire epidermis, including denuded areas, to support barrier function, reduce transcutaneous water loss, and encourage re-epithelialization 1, 2
• Regularly cleanse wounds by gently irrigating with warmed sterile water, saline, or dilute chlorhexidine 2
• Use appropriate dressings on exposed dermis to reduce fluid and protein loss, limit microbial colonization, help pain control, and accelerate re-epithelialization 1

Surgical Approach (Second-Line)

Consider surgical debridement of detached epidermis followed by biosynthetic xenograft or allograft application when conservative management fails, characterized by 1:

• Clinical deterioration
• Extension of epidermal detachment
• Local sepsis or subepidermal pus
• Delayed healing
• Wound conversion (progression to deeper cutaneous defect)

Infection Prevention and Management

Sepsis is the most common cause of death in SJS/TEN 1, 2. The exposed dermis becomes colonized initially by Staphylococcus aureus and later by Gram-negative organisms, especially Pseudomonas aeruginosa 1.

Critical infection management principles:

• Do NOT use prophylactic systemic antibiotics - indiscriminate administration increases skin colonization, particularly with Candida albicans 1
• Institute antimicrobial therapy only when clinical signs of infection are present 1
• Monitor carefully for systemic infection signs: confusion, hypotension, reduced urine output, reduced oxygen saturation, increased skin pain 1, 2
• Watch for laboratory indicators: rising C-reactive protein, neutrophilia, or monoculture on previously mixed-growth skin swabs 1, 2

Note that fever from SJS/TEN itself complicates detection of secondary sepsis 1, 2. Consider HSV reactivation in eroded or vesicular areas slow to heal, particularly in genital and oral sites 1.

Fluid Resuscitation

Fluid requirements in SJS/TEN are lower than those predicted by the Parkland formula used for burns - overaggressive resuscitation causes pulmonary, cutaneous, and intestinal edema 1.

• Establish adequate IV fluid replacement initially, guided by urine output and other end-point measurements 2
• Consider catheterization for accurate monitoring 2
• Avoid using burn formulas directly; titrate to clinical response 1

Nutritional Support

Provide continuous enteral nutrition throughout the acute phase 2:

• 20-25 kcal/kg daily during the early catabolic phase 2
• 25-30 kcal/kg daily during recovery 2

Pain Management

Use a validated pain assessment tool at least once daily to ensure adequate analgesia 2. Pain control is essential as denuded dermis is extremely painful and inadequate analgesia impairs patient cooperation with care 1.

Immunomodulatory Therapy Considerations

The evidence for specific immunomodulatory treatments remains controversial and lacks high-quality comparative studies 3, 4, 5. Options that have been used include:

• Systemic corticosteroids: The Indian guidelines recommend early (preferably within 72 hours) initiation of moderate to high doses (prednisolone 1-2 mg/kg/day or equivalent), tapered rapidly within 7-10 days 3
• Cyclosporine: 3-5 mg/kg/day for 10-14 days, either alone or in combination with corticosteroids 3, 5
• Intravenous immunoglobulin and anti-TNF agents have also been used 3, 5

However, high-quality studies assessing efficacy of immunomodulating agents in accelerating healing and reducing mortality are still lacking 4. The UK guidelines emphasize supportive care as the cornerstone without mandating specific immunomodulatory therapy 1.

Discharge Planning and Follow-Up

Before discharge 2:

• Provide written information about drugs to avoid
• Encourage wearing a MedicAlert bracelet to prevent future episodes
• Document drug allergy in patient's notes and inform all doctors involved in care
• Report the episode to national pharmacovigilance authorities

Long-term follow-up is essential as the most frequent sequelae are cutaneous, ocular, and psychological 4.

Common Pitfalls to Avoid

• Delayed transfer to specialized centers - this increases mortality 1
• Using prophylactic antibiotics - this promotes resistant organisms and fungal colonization 1
• Aggressive fluid resuscitation using burn formulas - this causes harmful edema 1
• Rough handling of skin - this extends epidermal detachment 1, 2
• Failure to involve ophthalmology early - ocular complications are common and require immediate specialized care 1