Díaz M S, Morita L, Ferrari B, Sartori S, Greco M F, Sobrevias Bonells L, González-Enseñat M A, Vicente Villa M A, Larralde M
Sector Dermatología Pediátrica, Hospital Ramos Mejía, Buenos Aires, Argentina.
Sector Dermatología Pediátrica, Hospital Ramos Mejía, Buenos Aires, Argentina.
Actas Dermosifiliogr (Engl Ed). 2019 Oct;110(8):673-680. doi: 10.1016/j.ad.2018.06.017. Epub 2019 Apr 20.
Linear IgA bullous dermatosis is an acquired subepidermal immunoglobulin-mediated vesiculobullous disease. In this retrospective, observational, descriptive study, we describe the clinical characteristics, treatments, and outcomes of 17 patients with linear IgA bullous dermatosis. Two children had been vaccinated 2 weeks before the onset of symptoms, 2 had had bronco-obstructive respiratory symptoms, and 1 had received intravenous antibiotic therapy. We also observed an association with autoimmune hepatitis in one patient and alopecia areata in another. One boy had VACTERL association. Diagnosis was confirmed by histopathology and direct immunofluorescence. Sixteen patients were treated with dapsone, which was combined with oral corticosteroids in 8 cases and topical corticosteroids in two. Of note in this series was the occurrence of relapses in the perioral area coinciding with infections and vaccination, and the association between linear IgA bullous dermatosis and autoimmune hepatitis and VACTERL association.
线状IgA大疱性皮肤病是一种获得性表皮下免疫球蛋白介导的水疱大疱性疾病。在这项回顾性、观察性、描述性研究中，我们描述了17例线状IgA大疱性皮肤病患者的临床特征、治疗方法及治疗结果。两名儿童在症状出现前2周接种了疫苗，两名有支气管阻塞性呼吸道症状，一名接受了静脉抗生素治疗。我们还观察到一名患者合并自身免疫性肝炎，另一名合并斑秃。一名男孩患有VACTERL综合征。通过组织病理学和直接免疫荧光检查确诊。16例患者接受了氨苯砜治疗，其中8例联合口服糖皮质激素，2例联合外用糖皮质激素。本系列研究值得注意的是，口周区域复发与感染和接种疫苗同时发生，以及线状IgA大疱性皮肤病与自身免疫性肝炎和VACTERL综合征之间的关联。
