Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome

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 Il 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.