Mori Francesca, Saretta Francesca, Liotti Lucia, Giovannini Mattia, Castagnoli Riccardo, Arasi Stefania, Barni Simona, Mastrorilli Carla, Pecoraro Luca, Caminiti Lucia, Marseglia Gian Luigi, Barbaud Annick, Novembre Elio
Allergy Unit, Department of Pediatrics, Meyer Children's University Hospital, Florence, Italy.
Pediatric Department, Latisana-Palmanova Hospital, Azienda Sanitaria Universitaria Friuli Centrale, Udine, Italy.
Front Pediatr. 2022 Jul 8;10:937528. doi: 10.3389/fped.2022.937528. eCollection 2022.
Linear Immunoglobulin A Bullous Disease (LABD) is a rare dermatosis whose pathomechanisms are not yet completely understood. LABD has different features characterizing adults and children in terms of potential triggers, clinical manifestations, and prognosis. The aim of the present study is to review all neonatal and pediatric cases of LABD and summarize the major characteristics. Childhood LABD is mainly idiopathic with a benign prognosis. Neonatal cases are difficult to differentiate from infectious diseases and usually have a poor prognosis. Drugs are one of the possible triggers that can activate autoimmune responses through antigen mimicry and epitope spreading as well as different stimuli (e.g., infections, inflammatory diseases, trauma). The gold standard for the diagnosis is based on direct immunofluorescence. Prognosis is generally favorable but often depends on the prompt dermatological diagnosis, treatment and follow-up guaranteed by a multidisciplinary team, including pediatricians for this group of age.
线状免疫球蛋白A大疱性疾病（LABD）是一种罕见的皮肤病，其发病机制尚未完全明确。LABD在潜在诱因、临床表现和预后方面具有不同的成人及儿童特征。本研究旨在回顾所有LABD的新生儿和儿科病例并总结其主要特征。儿童LABD主要为特发性，预后良好。新生儿病例难以与传染病相鉴别，通常预后较差。药物是可能的诱因之一，可通过抗原模拟和表位扩展以及不同刺激（如感染、炎症性疾病、创伤）激活自身免疫反应。诊断的金标准基于直接免疫荧光检查。预后通常良好，但往往取决于多学科团队（包括该年龄段的儿科医生）确保的及时皮肤病诊断、治疗及随访。
