Pediatric Toxic Epidermal Necrolysis

Abstract

Stevens Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare debilitating mucocutaneuous diseases most commonly associated with severe drug reactions. A review of SJS/TEN cases was undertaken to describe the etiology, patient characteristics, treatment, and outcome in children affected at a large tertiary pediatric hospital. A retrospective chart review examined SJS/TEN cases admitted to a pediatric intensive care unit (PICU) between 2001 and 2011. Data concerning total body surface area (TBSA) involvement, causative agents, length of stay, and treatment were reviewed. PICU mortality predictors including Severity-of-Illness Score for Toxic Epidermal Necrolysis (SCORTEN), Pediatric Index of Mortality 2 scores, and Pediatric Logistic Organ Dysfunction scores were also reviewed. Nutritional feeding information was collected and compared with calculated and measured (calorimetry) requirements. There were 10 SJS/TEN pediatric patients (mean age = 6.6 years) with significant skin involvement (mean TBSA = 42.2%) requiring PICU admission. Prescription drugs were the most common causative factor (n = 7). Intravenous immunoglobulin and corticosteroids were used for treatment in eight and two of the cases, respectively. The mean length of stay in the PICU was 10.2 days. There were no mortalities, and all mortality scores predicted high likelihood of survival. Actual feeds (n = 9) were uniformly lower than the calculated feeding requirements (mean = 1059 kcal/day vs mean = 2027 kcal/day). This data will help to provide insight into the management of SJS/TEN in an intensive care setting, and will help form the best treatment approach for future cases in a pediatric population.

Toxic Epidermal Necrolysis in Recessive Dystrophic Epidermolysis Bullosa following Bone Marrow Transplantation

“…Pediatric TEN is rare with few cases described in the literature (1,2), usually associated with anti-epileptic medications and antibiotics. While HCT has many well document risks, TEN within the context of HCT has only been described a handful of times (3,4), with roughly half of these cases later re-classified as TEN-like GvHD (Stage IV GvHD) (4).…”

“…In a recent case series of 36 children with TEN treated with IVIG, systemic corticosteroids, or a combination of both all patients survived (1). While adults with TEN in the setting of HCT have risk of mortality approaching 100% in some case series, it is unclear if this is also true in children (5).…”

Zespół toksycznej nekrolizy naskórka

“…No consensus standard treatment, particularly in children, has been established for SJS/TEN, which reflects the small number of patients studied [9,20,35]. An essential issue underscored in the literature is the need for early, rapid diagnosis, withdrawal of the causative agent inducing the inflammatory reaction, implementation of treatment and transfer of patients to specialised centres, which have been shown to decrease the incidence of complications and mortality rates [20].…”

“…According to Hamilton and colleagues [35], better prognostic factors of mortality in the paediatric population are the pediatric index of mortality (PIM 2) and pediatric logistic organ dysfunction (PELOD) commonly used in paediatric ICUs.…”