Rheumatology International

, Volume 32, Issue 11, pp 3643–3646 | Cite as

Juvenile systemic lupus erythematosus and dermatomyositis associated with urticarial vasculitis syndrome: a unique presentation

  • Patrícia A. Macêdo
  • Carolina B. Garcia
  • Monique K. Schmitz
  • Levi H. Jales
  • Rosa M. R. Pereira
  • Jozélio F. Carvalho
Case Report


To report a case of triple association of juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis and urticarial vasculitis as well as a review of the relevant literature. A 12-year-old male patient diagnosed with overlap syndrome between SLE and juvenile dermatomyositis since 2004 evolved with erythematous plaques, which were compatible with an urticarial rash. Clinical, laboratory and histopathological findings indicated a diagnosis of urticarial vasculitis. The patient previously had a C1q deficiency. Using the established treatment with methylprednisolone (1 g/day for 3 days), increasing doses of deflazacort and introduction of a dapsone, as well as mycophenolate mofetil regimen, with the suspension of azathioprine resulted in complete resolution of skin lesions. Urticarial vasculitis can present in various diseases. In SLE, presentation of urticarial vasculitis in children is rarely found. The triple association of juvenile-onset SLE, juvenile dermatomyositis and urticarial vasculitis is unusual, and this is the first case described in literature.


Systemic lupus erythematosus Dermatomyositis Urticarial vasculitis McDuffie syndrome 



We are grateful to Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPQ) #300559/2009-7 (to RMRP) and #300665/2009-1 (to JFC), Wilhelm Agrícola Federico Foundation Grant (to RMRP and JFC).


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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Patrícia A. Macêdo
    • 1
  • Carolina B. Garcia
    • 1
  • Monique K. Schmitz
    • 1
  • Levi H. Jales
    • 1
  • Rosa M. R. Pereira
    • 1
  • Jozélio F. Carvalho
    • 1
  1. 1.Rheumatology DivisionHospital das Clínicas da Faculdade de Medicina da Universidade de São PauloSão PauloBrazil

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