title: Childhood-onset bullous systemic lupus erythematosus.
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 Childreńs Institute of Hospital das Clínicas 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.
authors: Pediatric Rheumatology Unit, Pediatric Department, Faculdade de Medicina da Universidade São Paulo, São Paulo, Brazil.
authors: D M R
authors: DM
authors: Lourenço
authors: Pediatric Rheumatology Unit, Pediatric Department, Faculdade de Medicina da Universidade São Paulo, São Paulo, Brazil.
authors: R Cunha
authors: RC
authors: Gomes
authors: Pediatric Rheumatology Unit, Pediatric Department, Faculdade de Medicina da Universidade São Paulo, São Paulo, Brazil Division of Rheumatology, Faculdade de Medicina da Universidade São Paulo, São Paulo, Brazil.
authors: N E
authors: NE
authors: Aikawa
authors: Pediatric Rheumatology Unit, Pediatric Department, Faculdade de Medicina da Universidade São Paulo, São Paulo, Brazil.
authors: L M A
authors: LM
authors: Campos
authors: Department of Dermatology, Faculdade de Medicina da Universidade São Paulo, São Paulo, Brazil.
authors: R
authors: R
authors: Romiti
authors: Pediatric Rheumatology Unit, Pediatric Department, Faculdade de Medicina da Universidade São Paulo, São Paulo, Brazil Division of Rheumatology, Faculdade de Medicina da Universidade São Paulo, São Paulo, Brazil clovisaasilva@gmail.com.
authors: C A
authors: CA
authors: Silva
mesh_terms: D001768:Blister / Q000209:etiology / Q000473:pathology*; D001938:Brazil / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000453:epidemiology* / Q000473:pathology*; D008297:Male; D015995:Prevalence
chemical_list: D007136:Immunoglobulins
keywords: Bullous systemic lupus erythematosus; SLEDAI; children; dapsone