Bullous systemic lupus erythematosus in a 10-year-old child

Hans-Bittner, Nelise Ritter; Bittner, Guilherme Canho; Filho, Günter Hans; Takita, Luiz Carlos

doi:10.1590/abd1806-4841.20176074

Cited by 4 publications

(5 citation statements)

References 8 publications

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“…The current therapeutic regimens for BSLE are based on the pathogenesis of the disease, case-reports, and case-series. Dapsone is used as first-line therapy6 , 11 , 23 - 25 along with glucocorticoids and immunosuppressants. Other therapeutic options include mycophenolate mofetil5 , 7 , 13 , 26 methotrexate,27 rituximab,28 hydroxychloroquine and intravenous immunoglobulin 8…”

Section: Discussionmentioning

confidence: 99%

Bullous systemic lupus erythematosus a case report

et al. 2019

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Skin involvement in systemic lupus erythematosus (SLE) occurs in more than 75% of patients with this condition. Vesicles and blisters in lupus erythematosus (LE) may be present in SLE secondary to interface vacuolar changes in the epidermis, in discoid LE also secondary to vacuolar epidermal changes, and in bullous LE secondary to antibodies anti-collagen VII deposits with neutrophilic aggregates. In addition, blisters can occur due to the association of SLE with other autoimmune blistering diseases (e.g. bullous pemphigoid). BSLE is a rare blistering disease that mainly occurs in females (30–40 years old), and less frequently in children and adolescents. The most common presentation is rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques. Preferential sites are: superior trunk, proximal superior limbs, and face (lips) with symmetrical distribution. Mucosal involvement is common on perioral, pharyngeal, laryngeal, and genital areas. The involvement of sun-exposed areas is not mandatory. The lesions usually progress with no scarring, but hypo or hyperchromia may be present. We report an 18-year-old female patient with blistering lesions at admission, who was diagnosed with BSLE. She was initially treated with systemic prednisone and hydroxychloroquine. Her condition evolved with relapsing lesions, which required the introduction of Dapsone. The authors emphasize the relevance of recognizing BSLE—a rare presentation of SLE—which may evolve with marked clinical presentation.

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Section: Discussionmentioning

confidence: 99%

Bullous systemic lupus erythematosus a case report

et al. 2019

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“…60,61 The correlation between BSLE and systemic involvements including lupus nephritis, neuropsychiatric SLE and hematologic abnormality have been documented. 62 DIF studies of BSLE are usually performed in the perilesional area with clinical non-lesional skin to demonstrate staining of IgG, IgM, C3 and IgA at the DEJ with linear or granular patterns 35,59,[62][63][64][65][66][67][68][69][70][71][72][73][74]. The positive yield from the DIF test was high, at approximately 85.7%-100%.…”

Section: Non-specific Lupus Cutaneous Manifestations Bullous Systemic...mentioning

confidence: 99%

“…The positive yield from the DIF test was high, at approximately 85.7%-100%. 35,59,[62][63][64][65][66][67][68][69][70][71][72][73][74] IgG was the most reported immunoreactant, while IgA was more common in BSLE than other forms of lupus. 65 Furthermore, IgA deposits were associated with disease activity.…”

Section: Non-specific Lupus Cutaneous Manifestations Bullous Systemic...mentioning

confidence: 99%

Direct Immunofluorescence in Cutaneous and Systemic Lupus Erythematosus: A Literature Review

Rujitharanawong

Kulthanan²,

Tuchinda³

et al. 2023

Siriraj Med J

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Cutaneous manifestations of lupus erythematosus (LE) have a variety of clinical phenotypes and require proper investigation for diagnosis. Knowledge of the direct immunofluorescence (DIF) technique has improved and played an important role in the diagnosis of cutaneous LE. This review explores and summarizes reported DIF findings of each cutaneous LE variant. Historically, DIF findings of cutaneous LE have revealed deposits of multiple immunoreactants at the dermo-epidermal junction with either linear or granular patterns. Immunoglobulin M is the most common immunoreactant and DIF findings of cutaneous LE variants overlap. Therefore, diagnosis of cutaneous LE requires a combination of monitoring patient history, physical examinations and laboratory studies. This review helps interpret and better understand the application of DIF studies in cutaneous LE.

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“…La enfermedad ampollar puede ocurrir aislada o asociada a otras manifestaciones sistémicas de LES, particularmente nefritis y serositis. Por lo tanto, ante un LESB es mandatorio evaluar la presencia de compromiso sistémico asociado 15 , que en nuestro caso resultó ausente.…”

Section: Discussionunclassified

“…La dosis descrita de dapsona para el manejo del LESB varía entre 25 y 200 mg/día, con una dosis inicial recomendada de 50 mg/día 1 . La dosis en niños no está establecida, pero existen buenos resultados con dosis de 1,5 mg/kg/día 15 . Por lo tanto, tras el inicio de este fármaco, es posible aumentar la dosis de forma progresiva hasta lograr la respuesta esperada, como ocurrió en este caso.…”

Section: Discussionunclassified

Lupus eritematoso sistémico buloso: Una manifestación infrecuente en población pediátrica

Rollán

et al. 2021

Andes pediatr

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El lupus eritematoso sistémico buloso (LESB) es una enfermedad ampollar subepidérmica autoinmune secundaria a la presencia de autoanticuerpos contra el colágeno VII de la membrana basal. Es considerada una variante de lupus eritematoso sistémico (LES), siendo infrecuente en la población pediátrica.Objetivo: Describir caso de paciente pediátrica con erupción ampollar compatible con LESB.Caso Clínico: Paciente de sexo femenino de 16 años de ascendencia mapuche, con antecedentes de LES diagnosticado a los 10 años de edad, en tratamiento. Consultó por erupción vesiculobulosa generalizada de 6 semanas de evolución, sin sintomatología sistémica. Se realizó biopsia para estudio histológico e inmunofluorescencia directa (IFD), confirmándose el diagnóstico de LESB. La paciente respondió favorablemente al tratamiento con dapsona en dosis de 100 mg/día (asociado a su tratamiento de base), sin nuevas reactivaciones a 8 años de seguimiento.Conclusión: El LESB es una manifestación infrecuente de LES. Aunque la clínica es similar a la de otras dermatosis ampollares, la correlación entre la presencia de LES, los hallazgos histopatológicos y de IFD permiten confirmar el diagnóstico. Si bien se ha reportado mayor riesgo de LES en población de ascendencia indígena, faltan estudios respecto a la asociación de LESB en esta etnia.

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Bullous systemic lupus erythematosus in a 10-year-old child

Cited by 4 publications

References 8 publications

Bullous systemic lupus erythematosus a case report

Bullous systemic lupus erythematosus a case report

Direct Immunofluorescence in Cutaneous and Systemic Lupus Erythematosus: A Literature Review

Lupus eritematoso sistémico buloso: Una manifestación infrecuente en población pediátrica

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