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Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome
C Léauté-Labrèze1, T Lamireau, D Chawki
1Unité de Dermatologie Pédiatrique, Hôpital Pellegrin-Enfants, Place Amélie Raba-Leon, 33 076 Bordeaux Cedex, France.
Insights
Erythema multiforme (EM) and Stevens-Johnson syndrome (SJS) in children are often misdiagnosed and primarily triggered by infections, not drugs as seen in adults. Appropriate treatment leads to good outcomes.
Area of Science:
- Pediatric Dermatology
- Infectious Diseases
- Clinical Immunology
Background:
- Erythema multiforme (EM) in adults is typically linked to herpes infections.
- Stevens-Johnson syndrome (SJS) in adults is often associated with drug reactions.
- The etiology of EM and SJS in children requires further investigation.
Purpose of the Study:
- To test the hypothesis that EM and SJS have different triggers in children compared to adults.
- To review the management of pediatric patients diagnosed with EM or SJS.
- To analyze the causes and outcomes of EM and SJS in a pediatric cohort.
Main Methods:
- Retrospective analysis of 77 pediatric cases of EM or SJS.
- Inclusion criteria focused on documented cases between 1974 and 1998.
- Exclusion of inadequately documented or misdiagnosed cases.
Main Results:
- 42 pediatric patients with confirmed EM or SJS were analyzed.
- Childhood EM was predominantly linked to herpes infections.
- Childhood SJS was mainly associated with infectious agents, particularly Mycoplasma pneumoniae.
- Only two cases of SJS were attributed to antibiotics.
- No fatalities were recorded, and sequelae were minor.
- Corticosteroid treatment showed no significant overall benefit.
Conclusions:
- EM is frequently misdiagnosed in pediatric practice.
- The adult hypothesis of SJS being drug-related does not apply to children.
- Infectious agents are the primary triggers for both EM and SJS in children.
- Despite severe onset, EM and SJS have low morbidity and mortality with appropriate symptomatic care.
Background:
In adults, erythema multiforme (EM) is thought to be mainly related to herpes infection and Stevens-Johnson syndrome (SJS) to drug reactions.
Aims:
To investigate this hypothesis in children, and to review our experience in the management of these patients.
Methods:
A retrospective analysis of 77 paediatric cases of EM or SJS admitted to the Children's Hospital in Bordeaux between 1974 and 1998.
Results:
Thirty five cases, inadequately documented or misdiagnosed mostly as urticarias or non-EM drug reactions were excluded. Among the remaining 42 patients (14 girls and 28 boys), 22 had EM (11 EM minor and 11 EM major), 17 had SJS, and three had isolated mucous membrane involvement and were classified separately. Childhood EM was mostly related to herpes infection and SJS to infectious agents, especially Mycoplasma pneumoniae. Only two cases were firmly attributed to drugs (antibiotics). No patient died. EM and SJS sequelae were minor and steroids were of no overall benefit.
Conclusion:
In paediatric practice EM is frequently misdiagnosed. The proposal that SJS is drug related in adults does not apply to children, and in our recruitment EM and SJS are mostly triggered by infectious agents. The course of both diseases, even though dramatic at onset, leads to low morbidity and mortality when appropriate symptomatic treatment is given.