Childhood-onset bullous systemic lupus erythematosus

Author(s)

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. ^{[1, 2]}

Affiliation

^[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

• Document type: Journal article
• Source: Lupus; v. 23, n. 13, p. 1422-1425, NOV 2014.
• Web of Science Citations: 15

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 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)

• FAPESP's process: 11/12471-2 - Ovarian reserve, antibody anti-corpus luteum and lower genital tract infection in adult women with systemic lupus erythematosus, Behçet's syndrome and Takayasu's arteritis
• Grantee: Clovis Artur Almeida da Silva
• Support type: Regular Research Grants
• FAPESP's process: 08/58238-4 - Autoimmunity in children: investigation of the molecular and cellular bases of early onset of autoimmunity
• Grantee: Magda Maria Sales Carneiro-Sampaio
• Support type: Research Projects - Thematic Grants