Busca avançada
Ano de início
Entree
(Referência obtida automaticamente do Web of Science, por meio da informação sobre o financiamento pela FAPESP e o número do processo correspondente, incluída na publicação pelos autores.)

Childhood-onset bullous systemic lupus erythematosus

Texto completo
Autor(es):
Lourenco, D. M. R. [1] ; Gomes, R. Cunha [1] ; Aikawa, N. E. [2, 1] ; Campos, L. M. A. [1] ; Romiti, R. [3] ; Silva, C. A. [2, 1]
Número total de Autores: 6
Afiliação do(s) autor(es):
[1] Univ Sao Paulo, Fac Med, Dept Pediat, Pediat Rheumatol Unit, Sao Paulo - Brazil
[2] Univ Sao Paulo, Fac Med, Div Rheumatol, Sao Paulo - Brazil
[3] Univ Sao Paulo, Fac Med, Dept Dermatol, Sao Paulo - Brazil
Número total de Afiliações: 3
Tipo de documento: Artigo Científico
Fonte: Lupus; v. 23, n. 13, p. 1422-1425, NOV 2014.
Citações Web of Science: 15
Resumo

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 SAo 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. (AU)

Processo FAPESP: 11/12471-2 - Avaliação da reserva ovariana, do anticorpo anti-corpo lúteo e da infecção do trato genital inferior de mulheres adultas com lúpus eritematoso sistêmico, síndrome de Behçet e arterite de Takayasu
Beneficiário:Clovis Artur Almeida da Silva
Linha de fomento: Auxílio à Pesquisa - Regular
Processo FAPESP: 08/58238-4 - Autoimunidade na criança: investigação das bases moleculares e celulares da autoimunidade de início precoce
Beneficiário:Magda Maria Sales Carneiro-Sampaio
Linha de fomento: Auxílio à Pesquisa - Temático