Morbidity and Mortality of Mucocutaneous Diseases in the Pediatric Population at a Tertiary Care Center

Abstract

Toxic epidermal necrolysis (TEN) is a life threatening desquamating disease that is often an adverse reaction to drugs. Because mortality is so high, up to 30% nationally, and the morbidity significant, these cases are managed in burn centers. This study was conducted to evaluate what drugs were given to children who developed exfoliating skin disease and to identify the complications that these patients suffered. Thirty-two pediatric cases of erythema multiforme, Stevens-Johnson syndrome (SJS), and TEN were identified during a period of 8 years in which the average number of admissions to the burn center was 200 per year. Age, sex, drug history before admission, drug treatment during hospital stay, and clinical outcomes were noted. Several drugs were identified as probable causative agents. The most common cause of exfoliating disease was a combination of azithromycin and ibuprofen, followed by ibuprofen alone. Notably, the combination of ibuprofen and another drug was responsible for four additional cases, making the total percentage of pediatric cases involving ibuprofen 47%. Although no children died, several children with TEN and SJS suffered severe ocular involvement, sepsis, pneumonia, and genitourinary complications. All of the children who experienced complications had received ibuprofen. Chi-square analysis showed the correlation between ibuprofen and complications to be statistically significant (<0.05). This association was not observed with any other drug administered. Not only is ibuprofen a potential etiologic agent of exfoliating skin disease in children, it also may contribute to the development of complications in pediatric patients with the disease. Although this association does not prove that ibuprofen is the definitive cause of complications in these cases, caution is advised when giving this drug to children with suspected erythema multiforme, SJS, and TEN.

Lyell – szindróma gyermekkorban

“…Lefolyása kiszámíthatatlan, igen magas mortalitási arány jellemzô. Elôfordulási gyakorisága a teljes populációt tekintve 0.4-1.8:1000000, enyhe nôi túl-súly figyelhetô meg (1)(2)(3)(4)(5) (3)(4)(5)(6)(7)(8)(9)(10)(11). Patomechanizmusa nem teljesen tisztázott, azonban a gyógyszer, illetve gyógyszermetabolitok által beindított immunológiai reakció eredményeként aktiválódott specifikus cytotoxikus T-sejtek igen fontos szerepet játszanak a hámsejtek károsodásában, emellett a TNF-α, IFN-γ, IL-2 fokozott expressziója révén közvetített, illetve a Fas-FasL interakció révén létrejövô keratinocyta apoptosis is szerepet játszik benne (1, 2).…”

“…Az akut szemészeti ellátás során a lokális antibiotikumos készítmények megelôzik az infekciók kialakulását és a szteroid tartalmú szemcseppek csökkentik az akut gyulladást, ezek mellett szükség van megfelelô lubrikáns készítmények rendszeres használatára is (6,(12)(13)(14)16,17). A lubrikánsok jelentôs segítséget nyújthatnak a késôi szö-vôdmények megelôzésében és kezelésében is (11,12,15,16,18,19).…”

Zespół toksycznej nekrolizy naskórka

“…Several reports have described cases of SJS/TEN following the use of paracetamol [18]. The study involving 32 paediatric patients additionally showed that ibuprofen significantly worsened the course of disease and increased the risk of complications [19].…”

Pediatric Toxic Epidermal Necrolysis

“…In children, the incidence of SJS or TEN is even lower than adults. 7,8 While the mortality rate reported recently between 0 and 7.5% in pediatric SJS or TEN is less than in adults, [9][10][11][12] the importance of additional investigation into SJS or TEN specifically within the pediatric population should not be diminished as these children may suffer from potentially serious acute complications, 4 long-term sequelae, 7,13 and recurrence. 11 Given the rare and serious nature of SJS or TEN, there have been efforts to try to predict mortality in adults.…”