RESUMO
INTRODUCTION: Bullous systemic lupus erythematosus (BSLE) is an autoimmune subepidermal blistering disease se condary to the presence of autoantibodies against type VII collagen of the basement membrane zone. It is considered a variant of Systemic Lupus Erythematosus (SLE) and is uncommon in the pediatric population. OBJECTIVE: To describe the case of a pediatric patient with a bullous eruption compati ble with BSLE. CLINICAL CASE: A 16-year-old female patient of Mapuche descent with history of SLE diagnosed at age 10, undergoing treatment. She consulted due to a six-week history of a generalized bullous eruption with no systemic symptoms. Biopsy for histology and direct immunofluorescence (DIF) confirmed the diagnosis of BSLE. The patient responded favorably to dapsone 100 mg/day (associated with her baseline treatment), without new reactivations after 8 years of follow-up. Con clusion: BSLE is an infrequent manifestation of SLE. The clinical presentation is similar to other bullous dermatoses, but the histopathology and DIF in correlation with the presence of SLE confirm the diagnosis. Although indigenous ancestry is associated with SLE high-risk alleles, studies regarding the association of BSLE in this ethnic group are still lacking.
Assuntos
Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Dermatopatias Vesiculobolhosas/diagnóstico , Adolescente , Feminino , Humanos , Lúpus Eritematoso Cutâneo/patologia , Lúpus Eritematoso Sistêmico/patologia , Dermatopatias Vesiculobolhosas/patologiaRESUMO
La radiación ultravioleta ha sido usada durante décadas para el tratamiento de diversas enfermedades cutáneas. La radiación ultravioleta A1 (UVA-1) que tiene una longitud de onda entre los 340nm y 400 nm está disponible desde el año 1981, pero recién en las últimas dos décadas se ha estudiado, publicado y reportado su potencial uso terapéutico en la dermatología. Los primeros beneficios de su uso se reportaron en la dermatitis atópica donde se utilizaron dosis altas de UVA-1 para tratar las exacerbaciones severas de esta condición. Luego, nuevas indicaciones terapéuticas de su uso se fueron expandiendo a otras enfermedades cutáneas tales como: morfea, liquen escleroso, queratosis liquenoide, linfomacutáneo de células T y otras dermatopatías. La radiación UVA-1 al tener una longitud de onda más larga penetra a las capas más profundas de la dermis, lo que le permite una acción en la modificación de la respuesta inflamatoria, la respuesta inmunológica y los mecanismos de reparación cutánea.
Ultraviolet light radiation has been used for decades for the treatment of several cutaneous diseases. The ultraviolet radiation A1 (UVA-1) with a wave length between 340 nm-400 nm has been available since 1981, but only in the last two decades it has been studied and published for therapeutic use in dermatology. The first reported benefits of its use were reported in atopic dermatitis in which high doses of UVA-1were used to treat severe exacerbations of this condition. Thereafter, new therapeutic indications expanded its use for other cutaneous diseases like: morphea, lichen sclerosus, lichenoid keratosis, cutaneous T cell lymphoma and other skin conditions. The UVA-1 radiation has a long wavelength that make possible to reach the deep dermis and to modify the inflammatory response, immunological response and the cutaneous repair mechanisms.
