Management of severe toxic epidermal necrolysis in children

Toxic epidermal necrolysis (TEN) is a severe form of erythema multiforme th at results in extensive epidermal sloughing; the condition is associated wi th a mortality of up to 70%. From 1991 to 1998, 10 children with severe tox ic epidermal necrolysis were referred to a regional pediatric burn facility . Wounds were managed with strategy involving prevention of wound desiccati on and superinfection, including the frequent use of biologic wound coverin gs. Children unable to guard their airway because of extensive oropharyngea l involvement were prophylactically intubated. Enteral nutrition was stress ed. Steroids were not used and antibiotics were administered to managed spe cific foci of infection only. The 2 boys and 8 girls had an average age of 7.2 +/- 1.8 years (range 6 months to 15 years) and sloughed surface area of 76 +/- 6% of the body surface (range 50 to 95%). Antibiotics (3 children), anticonvulsants (3 children), nonsteroidals (2 children), and viral syndro me or unknown agents (2 children) were felt to have triggered the syndrome. Six children (60%) required intubation for an average of 9.7 +/- 1.8 days (range 2 to 14 days). Buccal mucosal involvement occurred in 9 (90%) and oc ular involvement in 9 (90%). Although infectious complications were common (2 pneumonias, 2 urinary infections, 1 bacteremia, 2 central line infection s, and 2 candidemias), all children survived after lengths of stay in the b urn unit averaging: 19 +/- 3 (range 6 to ) days. The most common long-term morbidity was keratitis sicca (2 children 20%), finger nail deformities (3 children, 30%), and variegated skin pigment changes (5 children, 50%). Alth ough having both a cutaneous and visceral wound that predispose them to inf ectious complications, most children with TEN will survive if managed with a strategy emphasizing biologic wound closure, intensive nutritional suppor t, and early detection and treatment of septic foci. Burn units have the re source set required to manage severe TEN and early referral of such childre n may have a favorable impact on survival.