Infantile Stevens Johnson syndrome and toxic epidermal necrolysis: A systematic review of clinical features and outcomes in children ages 12 months and under.

BACKGROUND/OBJECTIVES: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening mucocutaneous hypersensitivity reactions that carry significant morbidity and mortality. While clinical features are well documented in adult and pediatric patients, infantile cases are rarely reported. Our objective was to synthesize clinical features and outcomes in this population.

METHODS

A literature search was performed from three large databases (PubMed, EMBASE, and Web of Science) to systematically identify reports of SJS/TEN in the infantile period (defined as less than 12 months of age) between 1962 and 2019. Cases determined to represent SJS/TEN based on defined criteria were included. Each case was scored based on Quality Rating Scheme for Studies and Other Evidence. The initial search yielded 4856 publications, of which 19 (n = 26) met final inclusion criteria.

RESULTS

All cases for which body surface area (BSA) involvement was available or able to be approximated (n = 18/26) met criteria for TEN. All cases (n = 26) had mucous membrane involvement, with the oral mucosa most commonly affected (85.7%). Mortality was high within our population with 39.1% of infants expiring, 77.8% secondary to bacterial sepsis. The most common triggers were medications (52.4%), infections (33.3%), and vaccinations (14.3%).

CONCLUSIONS

This review highlights several unique clinical findings amongst infants with SJS/TEN, including increased BSA involvement, higher rates of bacterial sepsis, and higher mortality rates compared to older children and adults. Infants are more likely to present as TEN over SJS. More research is needed to identify triggers, successful treatments, and specific outcomes in this population.