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Chapter 30: Epidermal Necrolysis (Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis)

Maja Mockenhaupt

EPIDERMAL NECROLYSIS (STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS)

Therapeutic Approach

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, acute life-threatening mucocutaneous reactions characterized by extensive necrosis and detachment of the epidermis and mucosal epithelium. Because SJS and TEN share clinical pattern, histopathologic findings, etiology, risk factors, and mechanisms, they are considered as severity variants of one disease entity that differs only in the extent of skin detachment related to the body surface area. Identification and withdrawal of the culprit medication is part of the therapeutic concept in the majority of drug-induced cases, whereas appropriate management of infection is crucial for cases with infectious etiology. Therapy includes supportive care and topical and immunomodulating treatments, and aims to reduce morbidity, mortality, and long-term sequelae in survivors.1-3 Immunomodulating treatment may be started, when there is progression of the disease (new lesions not only detachment of old lesions) within the last 24 hours. If this is the case, it should be started without delay, unless there are strong contraindications. See Table 30-1.

Table 30-1Epidermal Necrolysis Treatment Table
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Table 30-1 Epidermal Necrolysis Treatment Table
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MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Supportive Care
Fluids, albumin, glucose Hypovolemia Fluid replacement for maintaining hemodynamic equilibrium; preferably via peripheral venous lines 2/3-3/4 of the recommendations for burns with similar extent of detachment; fluid volume in the first 24 h is 1.5 mL × % detached skin area × kg body weight Edema, if too much volume Needed IIb1,4,5
Electrolytes Electrolyte imbalance Correction of electrolyte imbalance As needed and recommended by protocols None, if monitored and adjusted Needed IIb1,4,5
Nutrition Calorie loss Promotion of healing and decreased risk of bacterial translocation from the gastrointestinal tract Early nutritional support via nasogastric tube; avoid parenteral nutrition; 20-25 kcal/kg/day during the first week, increase to 25-30 kcal/kg/day after the first week (ESPEN guidelines) None, if monitored and adjusted Needed IIb1,5,6
Morphine derivates, e.g. buprenorhine Pain Relief and prevention of pain, e.g. before skin care Morphine is indicated, if VAS score is ≥4/10   Needed III1,5,7
Heparine or derivatives Immobility Anticoagulation Prophylactic dosage     III1,5,7
Topical Therapy of the skin
Octenidine, lavanide, silver nitrate (“burn bath”), chlorhexidine solution Superinfection of the skin Disinfection of the skin May be warmed before application for patient comfort Rarely contact allergy   III1,5,7,8,9
Non-adhesive, non-medicated gauze dressings Denuded skin Avoidance of adhesion to sheets and other materials, enhancement of re-epithelialization Depending on the area of denuded skin None, when handled carefully   III1,5,7,8,9
Topical Therapy of the mucosa
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