TABLE 1

Summary of Cases of SJS/TEN-like LE in Pediatric Patients

AuthorAge, y/GenderPhoto-distributionMucous Membrane InvolvementPrevious LE DiagnosisPertinent Laboratory AbnormalitiesHistologyTherapy
Yu15/girlYesLipsYesANA 1:1280, increasing anti-dsDNA, decreasing complement levels, anemiaExtensive epidermal necrosis and subepidermal clefting. Interface dermatitis around pilosebaceous unit.Systemic steroids, intravenous IgG, mycophenolate mofetil, dapsone
Yu11/girlYesLips, ocular, vaginalYesANA 1:1280, anti-RNP positive, anti-Smith positive, increasing anti-dsDNA, decreasing complement levels, anemia, transaminitisNot availableSystemic steroids, intravenous IgG, plasmapheresis
Yu18/boyYesLipsYesIncreasing anti-dsDNA, decreasing complement levels, anemia, thrombocytopenia, transaminitis, elevated amylase and lipaseExtensive epidermal necrosis with sparse perivascular and interface lymphocytic dermatitisSystemic steroids, mycophenolate mofetil
Lee et al212/girlYesOral, lips, ocularNoANA >1:800, anti-dsDNA positive (trend unknown), anti-Smith positive, anti-Ro/SSA positive, anti-La/SSB positive, anti-RNP positive, hypocomplementemia (trend unknown), leukopenia, thrombocytopeniaBasal vacuolization with necrotic keratinocytes with perivascular lymphocytic infiltrateSystemic steroids and hydroxychloroquine
Jang et al316/girlYesLipsNoANA 1:640, anti-dsDNA positive (trend unknown), anti-Ro/SSA positive, anti-Smith positive, hypocomplementemia (trend unknown), leukopenia, anemiaSubepidermal clefting with interface dermatitis and marked necrosis of suprabasal keratinocytesSystemic steroids, topical steroids and hydroxychloroquine
  • RNP, ribonucleoprotein.