Childhood-onset bullous systemic lupus erythematosus

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dc.contributor.author Lourenco, D. M. R.
dc.contributor.author Gomes, R. Cunha
dc.contributor.author Aikawa, N. E.
dc.contributor.author Campos, L. M. A.
dc.contributor.author Romiti, R.
dc.contributor.author Silva, C. A.
dc.date.accessioned 2016-01-24T14:38:09Z
dc.date.available 2016-01-24T14:38:09Z
dc.date.issued 2014-11-01
dc.identifier http://dx.doi.org/10.1177/0961203314544187
dc.identifier.citation Lupus. London: Sage Publications Ltd, v. 23, n. 13, p. 1422-1425, 2014.
dc.identifier.issn 0961-2033
dc.identifier.uri http://repositorio.unifesp.br/handle/11600/38434
dc.description.abstract Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childres Institute of Hospital das Clinicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30-60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. the histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14-24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. in conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations. en
dc.description.sponsorship Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
dc.description.sponsorship Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
dc.description.sponsorship Federico Foundation
dc.description.sponsorship Nucleo de Apoio a Pesquisa Saude da Crianca e do Adolescente'' da USP (NAP-CriAd)
dc.format.extent 1422-1425
dc.language.iso eng
dc.publisher Sage Publications Ltd
dc.relation.ispartof Lupus
dc.rights Acesso restrito
dc.subject Bullous systemic lupus erythematosus en
dc.subject children en
dc.subject dapsone en
dc.subject SLEDAI en
dc.title Childhood-onset bullous systemic lupus erythematosus en
dc.type Artigo
dc.rights.license http://www.uk.sagepub.com/aboutus/openaccess.htm
dc.contributor.institution Universidade Federal de São Paulo (UNIFESP)
dc.contributor.institution Universidade de São Paulo (USP)
dc.description.affiliation Univ São Paulo, Fac Med, Dept Pediat, Pediat Rheumatol Unit, São Paulo, Brazil
dc.description.affiliation Univ São Paulo, Fac Med, Div Rheumatol, São Paulo, Brazil
dc.description.affiliation Univ São Paulo, Fac Med, Dept Dermatol, São Paulo, Brazil
dc.description.sponsorshipID FAPESP: 2008/58238-4
dc.description.sponsorshipID FAPESP: 2011/12471-2
dc.description.sponsorshipID CNPq: 302724/2011-7
dc.identifier.doi 10.1177/0961203314544187
dc.description.source Web of Science
dc.identifier.wos WOS:000344064900011



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