title: Juvenile systemic lupus erythematosus and dermatomyositis associated with urticarial vasculitis syndrome: a unique presentation.
abstract: 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.
authors.affiliation: Rheumatology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Avenida Dr Arnaldo 455, 3º andar, sala 3190, São Paulo 01246-903, Brazil.
authors.forename: Patrícia A
authors.initials: PA
authors.lastname: Macêdo
authors.forename: Carolina B
authors.initials: CB
authors.lastname: Garcia
authors.forename: Monique K
authors.initials: MK
authors.lastname: Schmitz
authors.forename: Levi H
authors.initials: LH
authors.lastname: Jales
authors.forename: Rosa M R
authors.initials: RM
authors.lastname: Pereira
authors.forename: Jozélio F
authors.initials: JF
authors.lastname: Carvalho
mesh_terms: D002648:Child; D003882:Dermatomyositis / Q000150:complications* / Q000473:pathology; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000473:pathology; D008297:Male; D013577:Syndrome; D014581:Urticaria / Q000150:complications* / Q000473:pathology; D014657:Vasculitis / Q000150:complications* / Q000473:pathology