Childhood-onset bullous systemic lupus erythematosus

Imagem de Miniatura
Arquivos
art_LOURENCO_Childhoodonset_bullous_systemic_lupus_erythematosus_2014.PDF (147.06 KB)
Citações na Scopus
30
Tipo de produção
article
Data de publicação
2014
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
SAGE PUBLICATIONS LTD
Autores
LOURENCO, D. M. R.
Citação
LUPUS, v.23, n.13, p.1422-1425, 2014
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
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.
Palavras-chave
Bullous systemic lupus erythematosus, children, dapsone, SLEDAI
URI
https://observatorio.fm.usp.br/handle/OPI/9043
Referências
  1. CAMISA C, 1983, J AM ACAD DERMATOL, V9, P924, DOI 10.1016/S0190-9622(83)70210-0
  2. Corrales IL, 2010, SEMIN CUTAN MED SURG, V29, P85
  3. Fernandez-Sanchez M, 2010, EUR J DERMATOL, V20, P823, DOI 10.1684/ejd.2010.1090
  4. Fujimoto W, 2005, J DERMATOL, V32, P1021
  5. Gammon WR, 1993, J INVEST DERMATOL, V100, P28
  6. Gladman DD, 2002, J RHEUMATOL, V29, P288
  7. Hochberg MC, 1997, ARTHRITIS RHEUM, V40, P1725, DOI 10.1002/art.1780400928
  8. Ludgate MW, 2008, AUSTRALAS J DERMATOL, V49, P91, DOI 10.1111/j.1440-0960.2008.00437.x
  9. Poojary S, 2012, CUTIS, V89, P17
  10. Saez-de-Ocariz M, 2010, PEDIATR RHEUMATOL, V8, DOI 10.1186/1546-0096-8-19
  11. Santos CE, 2013, REV BRAS REUMATOL, V53, P438
  12. Tincopa M, 2010, PEDIATR DERMATOL, V27, P373, DOI 10.1111/j.1525-1470.2010.01179.x
  13. Yung A, 2000, Australas J Dermatol, V41, P234, DOI 10.1046/j.1440-0960.2000.00426.x

Coleções
Artigos e Materiais de Revistas Científicas - FM/MPE
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - HC/ICr
Artigos e Materiais de Revistas Científicas - LIM/36