Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study

Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study

Author Sakamoto, A. P. Autor UNIFESP Google Scholar
Silva, C. A. Google Scholar
Saad-Magalhaes, C. Google Scholar
Alencar, A. N. Autor UNIFESP Google Scholar
Pereira, R. M. R. Google Scholar
Kozu, K. Google Scholar
Barbosa, C. M. P. L. Google Scholar
Terreri, M. T. Autor UNIFESP Google Scholar
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 Sao 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 were 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 antibio tics 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. Conclusion: Our study identified SJS and overlap SJS-TEN as rare manifestations of active cSLE associated with severe multisystemic disease, with potentially lethal outcome.
Keywords Stevens-Johnson syndrome
Toxic epidermal necrolysis
Childhood-onset systemic lupus erythematosus
Systemic lupus erythematosus
xmlui.dri2xhtml.METS-1.0.item-coverage Alges
Language English
Sponsor Conselho Nacional de Desenvolvimento Cientifico e Tecnologico
Federico Foundation
Nucleo de Apoio a Pesquisa "Saude da Crianca e do Adolescente" of USP (NAP-CriAd)
Grant number CNPq: 303422/2015-7
CNPq: 301805/2013-0
CNPq: 305068/2014-8
CNPq: 301479/2015
CNPq: 303752/2015-7
Federico Foundation
Date 2017
Published in Acta Reumatologica Portuguesa. Alges, v. 42, n. 3, p. 250-255, 2017.
ISSN 0303-464X (Sherpa/Romeo, impact factor)
Publisher Publisaude-Edicoes Medicas Lda
Extent 250-255
Access rights ACESSO ABERTO
Type Article
Web of Science ID WOS:000418501200006

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