Lúpus eritematoso sistêmico bolhoso na infância: relato de caso Bullous systemic lupus erythematosus in the childhood: case report

• Main Authors: Mariana Discacciati Chiminazzo (Author), Gracielle Cardoso (Author), José Roberto Pereira Pegas (Author), Valéria Aoki (Author), Ana Paula Galli Sanchez (Author)
• Format: Book
• Published: Sociedade Brasileira de Dermatologia, 2007-06-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 10.1590/S0365-05962007000300008; 0365-0596; 1806-4841
• Keywords: Criança; Dermatopatias vesiculobolhosas; Lúpus eritematoso sistêmico; Vesícula; Blister; Child; Skin diseases, vesiculobullous; Lupus erythematosus, systemic; Dermatology; RL1-803
• Source: Anais Brasileiros de Dermatologia, Vol 82, Iss 3, Pp 257-261 (2007)

É apresentado caso de lúpus eritematoso bolhoso na infância. Doente do sexo feminino, com nove anos, apresentava erupção vesicobolhosa no tronco, região cervical, genital e membros. O exame anatomopatológico mostrou bolha subepidérmica com neutrófilos, a imunofluorescência direta revelou depósito linear de IgA, IgM, IgG e C3 na zona da membrana basal, e a indireta foi negativa. Os anticorpos antinucleares e o anti-Sm estavam positivos. Houve regressão do quadro com dapsona e prednisona. Trata-se de caso raro de lúpus eritematoso sistêmico (LES) na infância que se iniciou com bolhas disseminadas. O LES bolhoso deve ser incluído no diagnóstico diferencial das erupções bolhosas na infância.<br>We report a case of bullous systemic lupus erythematosus in a 9-year-old female, with a vesiculobullous eruption on the trunk, neck, genitals and limbs. A skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed deposition of IgA, IgG, IgM and C3 at the epithelial basement membrane zone and indirect immunofluorescence was negative. Antinuclear antibodies and anti-Sm were positive. Resolution of the blisters occurred following treatment with dapsone and prednisone. This is a rare case of systemic lupus erythematous (SLE) in the childhood, whose initial manifestation was a bullous eruption. Bullous SLE should be considered in the differential diagnosis of children presenting with generalized bullous eruption.