Please use this identifier to cite or link to this item: https://repositorio.unifesp.br/handle/11600/38434
Title: Childhood-onset bullous systemic lupus erythematosus
Authors: Lourenco, D. M. R.
Gomes, R. Cunha
Aikawa, N. E.
Campos, L. M. A.
Romiti, R.
Silva, C. A.
Universidade Federal de São Paulo (UNIFESP)
Universidade de São Paulo (USP)
Keywords: Bullous systemic lupus erythematosus
children
dapsone
SLEDAI
Issue Date: 1-Nov-2014
Publisher: Sage Publications Ltd
Citation: Lupus. London: Sage Publications Ltd, v. 23, n. 13, p. 1422-1425, 2014.
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.
URI: http://repositorio.unifesp.br/handle/11600/38434
ISSN: 0961-2033
Other Identifiers: http://dx.doi.org/10.1177/0961203314544187
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