Characterization of Children With Recurrent Episodes of Stevens Johnson Syndrome

Abstract

We performed a retrospective chart review for all cases of recurrent Stevens Johnson Syndrome (SJS) from March 2013 to March 2016. Nine children had 29 episodes of SJS or incomplete SJS; all children were male and 8 (88%) were white. Episodes affected mucus membranes with minimal skin involvement. Mycoplasma infections and HLA-B27/-B51 were common.

Two pediatric cases of influenza B‐induced rash and mucositis: Stevens‐Johnson syndrome or expansion of the Mycoplasma pneumoniae‐induced rash with mucositis (MIRM) spectrum?

“…It is thought that some patients may have a genetic susceptibility to recurrent SJS. HLA‐B27 and HLA‐B51 have both been noted as predisposing factors to recurrent SJS . For Patient 1, testing was completed and was negative; however, this does not rule out the possibility of other loci associated with genetic susceptibility.…”

“…HLA-B27 and HLA-B51 have both been noted as predisposing factors to recurrent SJS. 12 For Patient 1, testing was completed and was negative; however, this does not rule out the possibility of other loci associated with genetic susceptibility. A prior study of pediatric SJS by Finkelstein et al found that up to 20% of cases of SJS in pediatric patients may have a recurrent course.…”

Recurrent erythema multiforme major in an 8-year-old patient with recurrence of herpetic gingivostomatitis and HLA-B*5801 haplotype: A causal or casual relationship?

“…Prognosis of EM major mainly depends on the extent of skin and mucosal involvement 2 and on a prompt treatment. Increasing evidence suggests a role of the immune system in the pathogenesis of EM reactions, but little was known about the predisposition to these reactions until recently 11, 12, 13…”

“…Malo et al 11 found that recurrent HSV-induced EM is strongly associated with the rare HLA allele DQB1*0402 in the disease form showing severe mucous membrane involvement. Recently, Olson et al 12 performed a retrospective chart review for cases of recurrent Stevens-Johnson syndrome in 9 children with mucous membrane–predominant phenotype, and HLA-B51 was found in 3 patients; HLA-B27 was found in 2 patients.…”

Severe Delayed Drug Reactions

“…38,39 Studies have reported that 14–63% of children with clinical SJS/TEN have detectable M. pneumoniae serum antibodies 40,41 and M. pneumoniae is recognized as a major trigger for re-current SJS episodes, particularly in children and adolescents. 40,42 SJS/TEN with eye involvement is more common in children with concurrent M. pneumoniae (26.7%) or HSV (15.6%) infections. 31 …”