Childhood-onset bullous systemic lupus erythematosus

Childhood-onset bullous systemic lupus erythematosus

Author Lourenco, D. M. R. Google Scholar
Gomes, R. Cunha Google Scholar
Aikawa, N. E. Google Scholar
Campos, L. M. A. Google Scholar
Romiti, R. Google Scholar
Silva, C. A. Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Universidade de São Paulo (USP)
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.
Keywords Bullous systemic lupus erythematosus
children
dapsone
SLEDAI
Language English
Sponsor Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
Federico Foundation
Nucleo de Apoio a Pesquisa Saude da Crianca e do Adolescente'' da USP (NAP-CriAd)
Grant number FAPESP: 2008/58238-4
FAPESP: 2011/12471-2
CNPq: 302724/2011-7
Date 2014-11-01
Published in Lupus. London: Sage Publications Ltd, v. 23, n. 13, p. 1422-1425, 2014.
ISSN 0961-2033 (Sherpa/Romeo, impact factor)
Publisher Sage Publications Ltd
Extent 1422-1425
Origin http://dx.doi.org/10.1177/0961203314544187
Access rights Closed access
Type Article
Web of Science ID WOS:000344064900011
URI http://repositorio.unifesp.br/handle/11600/38434

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