Clinical features, outcomes and treatment in children with drug induced Stevens-Johnson syndrome and toxic epidermal necrolysis

Clinical features, outcomes and treatment in children with drug induced Stevens-Johnson syndrome and toxic epidermal necrolysis

Authors

  • Lucia Liotti Department of Pediatrics, Senigallia Hospital, Senigallia, Italy
  • Silvia Caimmi Pediatric Clinic, Foundation IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
  • Paolo Bottau Pediatric and Neonatology Unit, Imola Hospital, Imola (BO), Italy
  • Roberto Bernardini Pediatric Unit, “San Giuseppe” Hospital, Empoli, Italy
  • Fabio Cardinale UOC di Pediatria, Azienda Ospedaliera-Universitaria “Consorziale-Policlinico”, Ospedale Pediatrico Giovanni XXIII, Bari, Italy
  • Francesca Saretta Pediatric Department, AAS2 Bassa Friulana-Isontina, Palmanova-Latisana, Italy; Pediatric Allergy Unit, Department of Medicine, Udine, Italy
  • Francesca Mori Allergy Unit, Department of Pediatric Medicine, Anna Meyer Children’s University Hospital, Florence, Italy
  • Giuseppe Crisafulli UO Allergologia, Dipartimento di Pediatria, Università di Messina, Italy
  • Fabrizio Franceschini UOC Pediatria, Azienda Ospedaliero-Universitaria “Ospedali Riuniti”, Ancona, Italy
  • Carlo Caffarelli Clinica Pediatrica, Dipartimento di Medicina e Chirurgia, Azienda Ospedaliero-Universitaria, Università di Parma, Italy

Keywords:

drug adverse reaction, Stevens-Johnson Syndrome, toxic epidermal necrolysis, hypersensitivity reactions, children, skin test, specific IgE, basophil activation test, drug provocation test

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), which can be considered a late-onset allergic reaction, can cause serious long-term sequelae. SJS/TEN are considered a spectrum of life-threatening adverse drug reactions. They have the same clinical manifestations and the only difference is in the extent of epidermal detachment. These conditions are associated with high mortality, although incidence of SJS/TEN is rare in children. SJS/TEN is an adverse drug reaction influenced by genes that involve pharmacokinetics, pharmacodynamics and immune response. Infective agents are additional influencing factors. Anticonvulsants and antibiotics, and especially sulphonamides and non-steroidal anti-inflammatory drugs, are among the drugs that were predominantly suspected of triggering SJS/TEN. No evidence-based standardized treatment guidelines for SJS or TEN are currently available. The usual treatment is mainly founded on the withdrawal of the suspected causative agent and supportive therapy. In pediatric patients, the specific therapeutic strategies are controversial and comprise systemic corticosteroids and the use of intravenous immunoglobulin (IVIG). More recently, new therapeutic approaches have been used, such as immunosuppressive therapies, including cyclosporine and TNF-α inhibitors.

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Published

29-01-2019

How to Cite

1.
Clinical features, outcomes and treatment in children with drug induced Stevens-Johnson syndrome and toxic epidermal necrolysis. Acta Biomed [Internet]. 2019 Jan. 29 [cited 2024 Apr. 14];90(3-S):52-60. Available from: https://www.mattioli1885journals.com/index.php/actabiomedica/article/view/8165

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