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

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

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.

MeSH terms

  • Acute Generalized Exanthematous Pustulosis / epidemiology*
  • Acute Generalized Exanthematous Pustulosis / etiology
  • Acute Generalized Exanthematous Pustulosis / therapy
  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Analgesics / therapeutic use
  • Anti-Bacterial Agents / adverse effects*
  • Anti-Inflammatory Agents, Non-Steroidal / adverse effects*
  • Anticonvulsants / adverse effects*
  • Child
  • Child, Preschool
  • Drug Hypersensitivity Syndrome / epidemiology*
  • Drug Hypersensitivity Syndrome / etiology
  • Drug Hypersensitivity Syndrome / therapy
  • Female
  • Hospitals, Pediatric
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Incidence
  • Infant
  • Intradermal Tests
  • Italy / epidemiology
  • Linear IgA Bullous Dermatosis / epidemiology
  • Linear IgA Bullous Dermatosis / etiology
  • Linear IgA Bullous Dermatosis / therapy
  • Lymphocyte Activation
  • Male
  • Patch Tests
  • Pemphigus / epidemiology
  • Pemphigus / etiology
  • Pemphigus / therapy
  • Retrospective Studies
  • Stevens-Johnson Syndrome / epidemiology*
  • Stevens-Johnson Syndrome / etiology
  • Stevens-Johnson Syndrome / therapy
  • Tertiary Care Centers

Substances

  • Adrenal Cortex Hormones
  • Analgesics
  • Anti-Bacterial Agents
  • Anti-Inflammatory Agents, Non-Steroidal
  • Anticonvulsants
  • Immunoglobulins, Intravenous
  • Immunologic Factors