What is the treatment approach for patients presenting with Toxic Epidermal Necrolysis (TEN), Erythema Multiforme (EM), or Stevens-Johnson Syndrome (SJS)?

Management of TEN, SJS, and Erythema Multiforme

Immediate Recognition and Differentiation

The critical first step is distinguishing these three conditions by body surface area (BSA) involvement and etiology: SJS affects <10% BSA, SJS/TEN overlap involves 10-30% BSA, and TEN affects >30% BSA, while erythema multiforme (EM) is a distinct entity primarily triggered by infections rather than drugs. 1, 2, 3

Key Diagnostic Features:

• SJS/TEN: Painful mucocutaneous erosions, hemorrhagic lesions, epidermal detachment with positive Nikolsky sign, typically drug-induced 1, 2
• Erythema Multiforme: Target lesions with central clearing, primarily infection-triggered (HSV, Mycoplasma pneumoniae), less severe mucosal involvement 4
• Document the exact date of symptom onset and calculate BSA involvement using Lund and Browder charts 1

Emergency Management for SJS/TEN

Immediate Actions (First 24 Hours):

Discontinue ALL suspected culprit drugs immediately—this is the single most critical intervention that determines survival. 1, 5, 6, 2

1. Calculate SCORTEN within 24 hours to predict mortality risk (0 parameters = 1% mortality; 5 parameters = 85% mortality) 1, 5, 7

2. Transfer patients with >10% BSA involvement to a specialized burn unit or ICU immediately—delayed transfer significantly increases mortality 1, 5, 8, 7

3. Establish IV access through non-lesional skin and begin fluid resuscitation guided by urine output, avoiding overaggressive hydration that causes pulmonary and intestinal edema 1, 5

4. Insert urinary catheter for accurate output monitoring and when urogenital involvement causes dysuria 1, 5

5. Obtain ophthalmology consultation within 24 hours—failure to do so results in permanent visual impairment 1, 5, 8, 7

Diagnostic Workup:

• Full blood count, electrolytes, liver/renal function, magnesium, phosphate, glucose, coagulation studies, Mycoplasma serology 1, 7
• Chest X-ray 1
• Skin biopsy from lesional skin adjacent to blister for histopathology showing full-thickness epidermal necrolysis 1, 7, 2
• Second biopsy from periblister skin for direct immunofluorescence to exclude immunobullous disorders 1, 7
• Bacterial and fungal swabs from lesional skin 1, 5

Specialized Supportive Care

Environmental Control:

• Barrier nursing in temperature-controlled room at 25-28°C on pressure-relieving mattress 5, 8, 7
• Control humidity to minimize transcutaneous water loss 5

Wound Management:

Handle skin with extreme care to minimize shearing forces—this prevents further epidermal detachment. 5, 8, 7

• Irrigate wounds gently with warmed sterile water, saline, or chlorhexidine (1:5000) 1, 5, 8
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis including denuded areas 1, 5, 8, 7
• Use non-adherent silicone dressings (Mepitel) to eroded areas 1, 5
• Apply secondary foam or burn dressings to collect exudate 1, 8
• Leave detached epidermis in situ as biological dressing 8
• Consider nanocrystalline dressings that can remain in place for longer periods 6

Fluid and Nutritional Management:

• Provide 20-25 kcal/kg daily during catabolic phase, 25-30 kcal/kg during recovery via continuous enteral nutrition 8, 7
• Insert nasogastric tube if oral intake precluded by buccal mucositis 8
• Monitor fluid balance carefully with regular vital signs, urine output, and electrolyte assessment 5, 7

Pain Management:

• Use validated pain assessment tools at least once daily 5
• Administer IV opioid infusions for patients unable to tolerate oral medication 5
• Consider patient-controlled analgesia 5
• Consider sedation or general anesthesia for dressing changes 5

Infection Prevention:

Do NOT use prophylactic systemic antibiotics—this increases skin colonization with resistant organisms, particularly Candida albicans. 5, 8, 7, 2

• Monitor for clinical signs of infection: confusion, hypotension, reduced urine output, decreased oxygen saturation 5
• Institute targeted antimicrobial therapy ONLY when clinical signs of infection present 5, 8, 7
• Obtain regular skin swabs for culture to detect predominant organisms 8, 7
• Watch for monoculture from multiple sites indicating invasive infection 5

Mucosal Management

Ocular Care (Critical):

Daily ophthalmology examination throughout acute phase is mandatory—neglecting eye care results in permanent visual impairment. 1, 5, 8, 7

• Apply preservative-free lubricant eye drops every 2 hours throughout acute illness 8, 7
• Daily ocular hygiene by ophthalmologist or trained nurse to remove inflammatory debris and break down conjunctival adhesions 8, 7
• Lyse adhesions immediately to prevent permanent symblepharon formation 8
• Apply topical antibiotics when corneal fluorescein staining or ulceration present 8
• Consider topical corticosteroid drops under ophthalmologist supervision 8
• Consider amniotic membrane transplantation in acute phase for better visual outcomes 8

Oral Care:

• Apply white soft paraffin ointment to lips immediately, then every 2 hours 8, 7
• Anti-inflammatory oral rinse (benzydamine hydrochloride) every 3 hours, particularly before eating 8
• Antiseptic oral rinse twice daily 8
• Topical anesthetics (viscous lidocaine 2% or cocaine mouthwashes 2-5%) for severe discomfort 8
• Treat candidal infection with nystatin 100,000 units four times daily for 1 week 8

Urogenital Care:

• Apply white soft paraffin ointment to urogenital skin and mucosae every 4 hours 8, 7
• Regular examination of urogenital tract during acute illness 8
• Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae 8

Systemic Immunomodulatory Therapy

If systemic therapy is initiated, it must be started within 72 hours of symptom onset to be effective. 8, 7, 4

Cyclosporine (Preferred Based on Evidence):

Cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month, has shown the most consistent benefit with reduced mortality compared to SCORTEN-predicted rates. 1, 8, 7, 4

• Multiple studies demonstrate benefit with standardized dosing 1
• May halt disease progression and lower mortality 4

Corticosteroids (Alternative):

• IV methylprednisolone 0.5-1 mg/kg daily if started within 72 hours of onset 8, 7
• Early pulse therapy may be beneficial but evidence remains debated 8, 7

Avoid:

• Routine use of IVIG lacks consistent evidence 2, 3

Multidisciplinary Team Coordination

Coordinate care through specialist skin failure team including dermatology/plastic surgery, intensive care, ophthalmology, and specialist skincare nursing. 1, 5, 7, 6, 2

Additional consultants as needed:

• Respiratory medicine for airway involvement 1
• Gastroenterology for esophageal involvement 2
• Gynecology for vaginal involvement 6
• Urology for urethral involvement 6
• ENT for nasopharyngeal involvement 6, 2
• Pain team 5
• Dietetics 5
• Physiotherapy 5

Management of Erythema Multiforme

EM requires supportive care only, as it is typically self-limited and infection-triggered rather than drug-induced. 4

• Identify and treat underlying infection (HSV, Mycoplasma pneumoniae) 4
• Symptomatic treatment with emollients and oral care 4
• No systemic immunosuppression required for uncomplicated EM 4
• Consider antiviral prophylaxis for recurrent HSV-associated EM 4

Discharge Planning and Long-Term Follow-Up

1. Provide written documentation of culprit drug(s) to avoid permanently, including cross-reactive medications 1, 8, 7

2. Encourage MedicAlert bracelet bearing culprit drug name 1, 8, 7

3. Document drug allergy in medical records and inform all healthcare providers 1, 8, 7

4. Report to pharmacovigilance authorities (Yellow Card Scheme in UK) 1, 8, 7

5. Arrange dermatology follow-up within weeks of discharge 1

6. Arrange ophthalmology follow-up within weeks if eye involvement occurred 1

7. Warn patients about fatigue and need for convalescence for several weeks 8

8. Consider referral to support groups (SJS Awareness UK) 8

Critical Pitfalls to Avoid

• Delayed transfer to specialized unit—single greatest modifiable risk factor for mortality 5, 8, 7
• Continued use of culprit medication—worsens condition and increases mortality 8
• Prophylactic antibiotics—increases resistant organism colonization 5, 8, 7
• Overaggressive fluid resuscitation—causes pulmonary, cutaneous, and intestinal edema 1, 5, 8
• Delayed ophthalmology consultation—results in permanent visual impairment 5, 8
• Failure to recognize sepsis—most common cause of death in acute phase 5, 2