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. 2020 Jan;50(1):61-73.
doi: 10.1111/cea.13513. Epub 2019 Oct 28.

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

Giulia Liccioli  1 , Francesca Mori  1 , Paola Parronchi  2 , Manuela Capone  2 , Lucia Fili  2 , Simona Barni  1 , Lucrezia Sarti  1 , Mattia Giovannini  1 , Massimo Resti  3 , Elio M Novembre  1
Affiliations

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

Giulia Liccioli et al. Clin Exp Allergy. 2020 Jan.

Abstract

Background: Severe cutaneous adverse reactions (SCARs) are delayed-type hypersensitivity reactions to drugs including as follows: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), Stevens-Johnson syndrome (SJS), Toxic Epidermal Necrolysis (TEN) and Acute Generalized Exanthematous Pustulosis (AGEP). Incidence, triggers and management of SCARs have not been investigated in large-scale epidemiological studies on children.

Objective: The aim of our study was to collect epidemiological, clinical and aetiological data from children with SCARs referred to our tertiary care paediatric hospital of Florence.

Methods: From 2010 to 2018 charts of children with diagnosis of SCAR were reviewed, and data collected during the acute phase and/or the subsequent allergy evaluation. Patients underwent patch tests, intradermal tests and lymphocyte transformation tests. All children were investigated for infectious diseases.

Results: Incidence of SCARs in hospitalized children was 0.32% over a 9-year period. Fifty-four children were enrolled (31 M; 23 F; median age 6.5 years): 17 cases of DRESS, 30 SJS, 3 TEN, 2 AGEP, 1 linear immunoglobulin A bullous disease (LABD) and 1 pemphigus. Twenty-eight out of 54 patients underwent drug allergy investigations, and 50% of them resulted positive. Combining clinical history and results of allergy work-up, 74% SCARs seem to be caused by drugs, 18.6% by both drugs and infections, 3.7% by infections, and 3.7% remained idiopathic. No deaths occurred.

Conclusions: In this study, SCARs incidence is in line with literature data. Drugs were most commonly the leading cause. Management of SCARs requires cooperation among professional figures for an early diagnosis and a prompt treatment. Mortality rate seems to be lower in children.

Keywords: Stevens-Johnson syndrome; acute generalized exanthematous pustulosis; aetiopathogenesis; children; drug reaction with eosinophilia and systemic symptoms; hypersensitivity drug reactions; lymphocyte transformation tests; paediatrics; severe cutaneous adverse reactions; toxic epidermal necrolysis.

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References

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