title: Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study.
abstract: OBJECTIVE
  To assess Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in a large population of childhood-onset systemic lupus erythematosus (cSLE) patients.
  
  
  METHODS
  Multicenter study including 852 cSLE patients followed in Pediatric Rheumatology centers in São Paulo, Brazil. SJS was defined as epidermal detachment below 10% of body surface area (BSA), overlap SJS-TEN 10-30% and TEN greater than 30% of BSA.
  
  
  RESULTS
  SJS and TEN was observed in 5/852 (0.6%) cSLE female patients, three patients were classified as SJS and two patients were classified as overlap SJS-TEN; TEN was not observed. The mean duration of SJS and overlap SJS-TEN was 15 days (range 7-22) and antibiotics induced four cases. Regarding extra-cutaneous manifestations, hepatomegaly was observed in two cSLE patients, nephritis in two and neuropsychiatric involvement and conjunctivitis were observed respectively in one patient. Hematological involvement included lymphopenia in four, leucopenia in three and thrombocytopenia in two patients. The mean SLEDAI-2K score was 14.8 (range 6-30). Laboratory analysis showed low C3, C4 and/or CH50 in two patients and the presence of anti-dsDNA autoantibody in two patients. One patient had lupus anticoagulant and another one had anticardiolipin IgG. All patients were treated with steroids and four needed additional treatment such as intravenous immunoglobulin in two patients, hydroxychloroquine and azathioprine in two and intravenous cyclophosphamide in one patient. Sepsis was observed in three cSLE patients. Two patients required intensive care and death was observed in one patient.
  
  
  CONCLUSIONS
  Our study identified SJS and overlap SJS-TEN as rare manifestations of active cSLE associated with severe multisystemic disease, with potentially lethal outcome.
authors.affiliation: Universidade Federal de São Paulo (UNIFESP).
authors.forename: Ana Paula
authors.initials: AP
authors.lastname: Sakamoto
authors.affiliation: Faculdade de Medicina da Universidade de São Paulo (USP).
authors.forename: Clovis Artur
authors.initials: CA
authors.lastname: Silva
authors.affiliation: São Paulo State University (UNESP) - Faculdade de Medicina de Botucatu.
authors.forename: Claudia
authors.initials: C
authors.lastname: Saad-Magalhães
authors.affiliation: Universidade Federal de São Paulo (UNIFESP).
authors.forename: Aline Nicácio
authors.initials: AN
authors.lastname: Alencar
authors.affiliation: Faculdade de Medicina da Universidade de São Paulo (USP).
authors.forename: Rosa Maria Rodrigues
authors.initials: RMR
authors.lastname: Pereira
authors.affiliation: Faculdade de Medicina da Universidade de São Paulo (USP).
authors.forename: Katia
authors.initials: K
authors.lastname: Kozu
authors.affiliation: Hospital Infantil Darcy Vargas.
authors.forename: Cassia Maria Passarelli Lupoli
authors.initials: CMPL
authors.lastname: Barbosa
authors.affiliation: Universidade Federal de São Paulo (UNIFESP).
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Terreri
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D012189:Retrospective Studies; D013262:Stevens-Johnson Syndrome / Q000150:complications*