Clinical Features and Treatment Outcomes among Children with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A 20-Year Study in a Tertiary Referral Hospital

Abstract

Aim To determine the probable causative factors, clinical features, and treatment outcomes of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS-TEN overlap in children. Methods A 20-year database review of all children diagnosed with SJS/TEN/SJS-TEN overlap at the King Chulalongkorn Memorial Hospital, Thailand. Results 36 patients (M : F, 16 : 20) with the mean age of 9.2 ± 4.0 years were identified. There were 20 cases of SJS, 4 cases of SJS-TEN overlap, and 12 cases of TEN. Drugs were the leading cause for the diseases (72.3%); antiepileptics were the most common culprits (36.1%). Cutaneous morphology at presentation was morbilliform rash (83.3%), blister (38.9%), targetoid lesions (25.0%), and purpuric macules (2.8%). Oral mucosa (97.2%) and eye (83.3%) were the 2 most common mucosal involvements. Majority of the cases (77.8%) were treated with systemic corticosteroids, intravenous immunoglobulin, or both. Treatment outcomes between those who received systemic therapy and those who received only supportive care were comparable. Skin and eye were the principal sites of short-term and long-term complications. Conclusions SJS/TEN are not common but are serious diseases which lead to significant morbidities in children. Early withdrawal of suspicious causes and meticulous supportive care are very important. This study found that the systemic therapy was not superior to supportive care because the treatment outcomes for both groups were comparable.

Aetiopathogenesis of severe cutaneous adverse reactions (SCARs) in children: A 9‐year experience in a tertiary care paediatric hospital setting

“…In our case series of paediatric patients only two, both with TEN, required intensive care and none died, confirming that mortality rate from SCARs is higher in adults than in children, probably because of different immunological response and comorbidity of chronic diseases that are rare in paediatric patients.…”

“…This association was successful, as our children suffered no sequelae after overcoming the acute phase. Almost all the paediatric studies on SJS/TEN have reported that CS, alone or in combination with IVIg, are the most important treatments, in addition to antimicrobics and supportive care, to ensure a low mortality rate and the use of CS does not seem to associate with an increased risk of infections …”

“…For example, SJS may be induced by infectious agents ( Mycoplasma pneumoniae and viral infections) in up to 30% cases . Taking into account the main studies on SJS/TEN, about 16% of cases remain idiopathic, without any history of drug intake, or any infections detected . In the present study, we included other bullous reactions, not belonging to the SCAR group: linear immunoglobulin A bullous disease (LABD), a widespread bullous eruption with typical annular disposition and evidence of linear deposition of IgA at the dermoepidermal junction under immunofluorescence and pemphigus.…”

“…In a recent review, Adler et al summarize three major algorithms to assess drug causality in SCARs: the Naranjo scale, the Algorithm of Drug Causality for Epidermal Necrolysis (ALDEN) for SJS/TEN, the European Registry of Severe Cutaneous Adverse Reactions to Drugs (RegiSCAR) criteria, the Japanese group of Severe Cutaneous Adverse Reactions to Drugs (SCAR‐J) criteria for DRESS, the European Study of Severe Cutaneous Adverse Reactions scoring system for AGEP . In literature, the rate of hospital admissions for SCARs is reported to be from 0.3 up to 3.6/1000 and many epidemiological studies focused on SCARs analysed incidence, demographic characteristics of patients, possible triggers and mortality rate in a hospital setting . In most of them, diagnosis was based on clinical features and history of drug administration, without allergy investigation, except for a few .…”

A Case Report: Toxic Epidermal Necrolysis in Children

“…The patients presented with morbilliform rash (83.3%), blisters (38.9%), targetoid lesions (25.0%), and purpuric macules (2.8%), and the most common mucosal involvements were oral (97.2%) and eye mucosae (83.3%). 9 Lesions in this patient's mucosae followed the same order as those reported in the literature, starting at the oropharynx, and spreading to the ocular, genital, and anal mucosae. 10 In 2000 was published an article with a toxic epidermal necrolysis-specific severity-of-illness score for adults le to predict prognosis for the epidermal necrolytic disorders, called SCORTEN.…”